UWORLDS Flashcards

(720 cards)

1
Q

where are these seen? are they all from the same disease?

A

NERP!

ALZHEIMERS.

A= Senile plaques in gray matter: extracellular

β-amyloid core; may cause amyloid angiopathy intracranial hemorrhage; Αβ (amyloid-β) synthesized by cleaving amyloid precursor protein (APP).

ALZHEIMERS STILL

B= Neuro brillary tangles: intracellular, hyperphosphorylated tau protein = insoluble cytoskeletal elements; number of tangles correlates with degree of dementia.

Frontotemporal dementia

C=Inclusions of hyperphosphorylated tau (round

Pick bodies; (can also see ubiquitinated TDP-43)

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2
Q

this Congenital heart defect is:

*Caused by anterosuperior displacement of the infundibular septum.

*Is the Most common cause of early childhood cyanosis.

whats the disease? what are the 4 main features?

A

Tetrologoy of Fallot

Pulmonary infundibular stenosis (most important determinant for prognosis)

Right ventricular hypertrophy (RVH)— boot-shaped heart on CXR

Overriding aorta

VSD (harsh systolic murmur)

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3
Q

Clinical syndrome characterized by acute onset respiratory failure, bilateral lung opacities “white out on CXR), LOW Pao2/Fio2, _no evidence of HF/ fluid overload.= NORMAL PCWP._

whats the diagnosis and what is characteristically seen on histo?

A

ARDS

Intra-alveolar hyaline membranes

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4
Q

what the MOA behind the AE of osteoporosis caused by corticosteroid use

A
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5
Q

man has a painless scrotal mass

no transillumination

PALPABLE WHEN STANDING BUT DISAPPEARS WHEN LAYING RECUMBANT

whats the Dx?

A

VARICOCELE

note: testicular cancer, spermatocele, testicular torsion DO NOT change in size when laying and although a communicating HYDROCELE (patent process vaginalis) DOES change in size it TRANSILLUMINATES SO varicocele is the only option

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6
Q

know how to seperate wernicke encephalopathy from korsakoff syndrome.

wernicke you get, for eaxmple. from thiamine def and it has a triad of

  1. ataxia
  2. confusion
  3. oculomotor dysfuntion

that resolves when given thiamine

KORSAKOFF is A COMPLICATION OF WERNICKE AND its hallmarks are PERMAMENT MEMORY LOSS AND CONFABULATION!

A
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7
Q

describe the extensive step wise tx of RA

A

NSAIDs, glucocorticoids,

use as a BRIDGE TO

disease-modifying agents ((DMARDS)

  1. methotrexate
  2. sulfasalazine,
  3. hydroxychloroquine,
  4. leflunomide

and when DMARDS dont work try: TNF-a inhibitors

  1. Etanercept -(Fusion protein (receptor for TNF-α + IgG1 Fc), produced by recombinant DNA. its a tnf decoy receptor
  2. Infiximab & adalimumab [Anti-TNF-α monoclonal antibody]
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8
Q

whats the MOA of diphendyramine of Dimenhydrinate or Meclizine?

A

1st gen: Reversible inhibitors of H1 histamine receptors (can cross BBB).

used for

  1. Allergy
  2. motion sickness (THIS IS DUE TO THEIR Antagonist effect at muscarinic-3 (M3) receptors)
  3. b (bc since they cross the BBB and have CENTRAL effects too, they cause drowsiness)
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9
Q

which Plasmodium class has an exoerythrocytic (dormant form (hypnozoite) in liver) form?

and how would you treat this?

is there a particular AE to look out for ?

A

For P vivax/ovale (not falciparum),

this how you would treat

Chloroquine (for sensitive species), which blocks Plasmodium heme polymerase;

if resistant, use mefloquine or atovaquone/ proguanil

If life-threatening, use intravenous quinidine or artesunate (test for G6PD de ciency)

AAAAND FINALLY TO ANSWER THE ACTUAL Q:

add primaquine for the exoerythrocytic (dormant form (hypnozoite) in liver)

(test for G6PD de ciency)!!!!!!

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10
Q

17 y/o boy

non blanching palpable purpura after taking penicilin

on biopsy what wil you see?

A

leukocytoclastic (cutaneous small vessel vasculitis) vasculitis!!!! (shows fragmented neutrophili nuclei + inflamed smal bv’s with Fibrinoid necrosis)

this only affects the skin and normally arises from exposure to a drug or pathogen (Hep B or C virus or penicillin, cephalsporin)

similar to what is seen in in HENOCH (leukocytoclastic angitis + IgA + C3)

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11
Q

which 2 vasculitis’ give you +ve p-ANCA

A

Microscopic Polyangitis & Churg-Strauss (eosinophilic granulomatosis with polyangitis)

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12
Q
A
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13
Q

Does COX-1 or COX-2 protect the GI mucosa? and how? explain which SPECIFIC PGs are made from each pathway and their diff fxs.

A

COX-1 (from PLTS) makes TXA2 + PGI2 and E2 where as

COX-2 (from ENDOTHELIUM) two makes PGI2

PGE2 is PROTECTIVE TO GI MUCOSA SO COX-1 IS MORE PROTECTIVE TO GI MUCOSA.

note: PGI2 is in charge of 1. dec plt. aggregation 2. vasodilation

where as COX-1 makes TXA2 which opposes (or balances) PGI2 by promoting PLT aggregation + vasoCONSTRICTION

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14
Q

where is the biggest O2 content difference when compared to the Aorta (that has High O2 content)?

pick one

Pulmonary Artery

IJV

Hepatic Vein

Coronary Sinus

Brachial Vein

A

you may want to say Pulmonary Artery BUT NO! answer is = CORONARY SINUS

the coronary sinus receives blood from the cardiac veins which are the most DEOXYgenated (O2 poor) veins in the body bc EXTRACTION/use/sucking up of O2 in the heart is the greatest out of all the veins in the body (eg: skeletal muscle) so since the CORONARY SINUS receives blood from the cardiac vein and gives it to the RA, IT IS THE PLACE THAT RECEIVES THE LEASE OXYGENATED BLOOD.

AGAIN, why not the PA?

bc it receives blood from the Coronary sinus (via the right hear) ANDDDD also from the vena cava which is MORE oxygenated than the plain ‘ol cardiac veins so since Coronary sinus JUST received SUPER SUPER DEOXY BLOOD IT IS THE PLACE THAT DIFFERS THE MOST FROM THE AORTA AS FAR AS O2 CONTENT.

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15
Q

main mode of transmission of HEP A?

A

Blood!!!!!

(IVDU, post- transfusion)

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16
Q

how do the levels of absorption and hence levels of Dxylose measured in blood & urine CHANGE in

  1. pancreatic insufficiency or removal of panc
  2. GI mucosal defects
  3. bacterial overgrowth
A
  1. NO CHange! if panc messed up it can still be absorbed bc dxylose is a MONOsaccharide (like galactose and gllucose) so they can be absorbed at proximal SI by either fac diffuusion or the SGLT1

the other 2 would DECREASE levels of dxylose seen in urine and blood

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17
Q

which disease has the following characteristics:

female of reproductive age

Verrucous (Libman-Sacks) Endocarditisnonbacterial, verrucous thrombi usually on mitral or aortic valve. (DESCRIBE THE VEGETATIONS SEEN on the valve leaflets)

Associated with diffuse proliferative glomeruloneprhitis (proliferative and necrotizing lesions with crescent formation during active disease)

-what does this light microscopy look like? (looking for a buzz word here)

A

SLE

vegetations are seen on BOTH surfaces of valve leaflets and are composed of STERILE platelet thrombi interwined with strands of fibrin, immunecomplexes and mononuclear cells. *they are easily dislodged and can result in systemic embolization.

LM: diffuse thickennig of the glomerular capillary walls with “WIRE LOOP” structures due to subendothelial immune complex deposition”

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18
Q

after passing the inguinal ligament, the external iliac artery becomes the_______

A

Common femoral artery

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19
Q

What accounts for HCVs genetic instability during the replication process?

lack of 3-5’ exonuclease activity

or

lack of 5-3’ exonuclease actiivity?

A

they vary at sequences encoding for its 2 envelope glycoproteins

lacks 5-3’ exonuclase activity!!!!

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20
Q

pt with

RUQ pain (acute onset) + nausea + vomit

receiving TPN (total parenteral nutrition)

moderate leukocytosis

gallstones seen on ultrasound

why/how does TPM lead to the complication of Gallstones?

qID: 77

A

normally, enteral passage of fatty acids & amino acids into the duodenum–> +release of CCK–> contraction of gall bladder.

if TPN=no enteral stimuation–> no CCK released–> BILIARY STASIS–> INC RISK OF GALL STONES

side note: resection of the ileum ca also cause inc risk of gall stones due disruption of normal enterohepatic circulation of bile acids

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21
Q

PATIENT HAS SEPSIS AND SHOCK after pnm and after three days his hb levels drop and nasogastric suction shows bright read blood?

why?

A

bc THE SHOCK AND SEPSIS DECREASES BLOOD FLOW TO MUCOSA AND THIS LEADS TO “STRESS RELATED MUCOSAL INJURY!!”

LIKE HELLO…THIS IS ACUTE GASTRITIS!! EROSIONS AND THEY CAN ALSO PERFORATE.

REMEMBER WE think of nsaids and cushings and curling ulcers but SHOCK is a huge one bc our stomach NEEDS BLOOD AS A PROTECTIVE BARRIER!!!!!!!!

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22
Q

27-year-old primigravid woman at 18 weeks gestation comes to physician for routine prenatal examination. The uterus consistent in size with 18 week gestation. Ultrasonography shows a male fetus. The collecting system and pelvis of the left kidney is dilated and the renal cortex appears compressed.

The left and right ureters are not dilated. The right kidney appears normal. Amniotic fluid volume is normal. Which causing renal finding in this fetus ?

A

Incomplete recanalization of proximal ureter

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23
Q

a patient with a taenia infection in the intestine or (intestinal tapeworm) or Cysticercosis (eg: in muscle) would be treated with Praziquantel

BUT

neurocysticercosis is treated with….

A

albendazole !!!!!! for neurocysticercosis

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24
Q

t(8;14)—translocation of c-myc (8) and heavy-chain Ig (14)

t(14;18)—translocation of heavy-chain Ig (14) and BCL-2 (18)

t(11;14)—translocation of cyclin D1 (11) and heavy-chain Ig (14)

A

t(8;14)—translocation of c-myc (8) and heavy-chain Ig (14)———–Burkitt lymphoma

t(14;18)—translocation of heavy-chain Ig (14) and BCL-2 (18)———-Follicular lymphoma

t(11;14)—translocation of cyclin D1 (11) and heavy-chain Ig (14) ——–Mantle cell lymphoma

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25
anti-U1 RNP (speckled ANA) antibodies are associated with....
MIXED CONNECTIVE TISSUE DISEASE
26
diff btw libman sacs endocarditis and bacterial endocarditis?
LS endo has vegetations composed of sterile platelet thrombi with immune complexes (SMALL) vegetations on both surfaces of valve)-mitral and aortic Bacterial endocarditis has LARGE vegetations on valve cusps that contain inflammatory cells and bacteria (staph or strep) both contain fibrin
27
homeless alcholic man ingests ethylene glycol and 24-72 hours later will manigest with signs of acute renal failure bc he has ATN....what is his acid base status? WHATS THE ANTIDOTE FOR ETHYLENE GLYCOL? MOA? IS THIS ANTIDOTE USED FOR ANYTHING ELSE?
**HIGH ANION GAP** METABOLIC ACIDOSIS **FOMEPIZOLE: INHIBITS ALCOHOL DH** **(cant go from ethanol to acetaldehyde)** **can also use for METHANOL INTOX**
28
Nf kappa beta normally does what and what drug class inhibiits it classically?
Nf kappa beta normally upregulates synthesis of inflammatory proteins Corticosteroids
29
The pathognomonic microscopic feature in this disease is the **GRANULOMATOUS TISSUE CONTAINING lipid-laden foamy macrophage**
**Xanthogranulomatous Pyelonephritis**
30
31
what are the **3 main complications** associated with the disease that is confirmed when **Asbestos (ferruginous) bodies** are golden-brown fusiform rods resembling dumbbells are found in alveolar septum sputum sample, visualized using **Prussian blue stain**, often obtained by **bronchoalveolar lavage.**
Risk of **1.** **bronchogenic carcinoma** **\>\> 2.** risk of mesothelioma. **3. risk of pleural effusions.**
32
what oxidase +ve bug causes Contact lens keratitis? and whats the property of this bug that enables this?
pseudomonas can form a **biofilm**
33
4 main manifestations of **Protein malnutrition-----Kwashiorkor**
Kwashiorkor results from a protein-deficient (carbs\>protein) BUT total caloric intake is NORMAL (unlike marasmus) kid with big belly **(ASCITES!) + dec CMI (inc risk for parasites) +Apathy, listlessness, poor appetite --\> POOR PROGNOSIS!!** MEAL: **Malnutrition** Edema **(pittng)** **Anemia:** RBC hypoplasia; iron/ folic acid/vitamin B12 deficiencies **Liver (fatty)**--(due to dec apolipoprotein synthesis..NEED APO B100 FOR vldl to leave liver)--\> **massive hepatomegaly** dont confuse with **Marasmus!** which is **Total calorie malnutrition** resulting in emaciation (tissue and muscle wasting, **loss of subcutaneous fat);** +/– edema.
34
HYPOPLASTIC THYMUS (absent thymic shadow) is seen in...name 2 diseases
DI GEORGE (low Ca2+) SCID (normal Ca2+)
35
fibrinoid necrosis is seen in what two disease processes
1. Vasculitis (eg: PAN) 2. Malignant HTN
36
*whats the diagosis? whats the genetic principle underlying this disease?* **hyperphagia,** **obesity**, **intellectual disability (mental retardation)**, hypogonadism, and hypotonia, almond shaped eyes, temperature instability, balanced translocation at chr. 15 this disease is due to deleted/mutated genes on CHROMOSOME 15!!!!
**PRADER WILLI syndrome!** IMPRINTING!!!! At some loci, only one allele is active; the other is **inactive (imprinted/inactivated by methylation).** With one allele inactivated, deletion of the active allele disease. 25% of cases due to **maternal uniparental disomy** (two maternally imprinted genes are received; **no paternal gene received).**
37
proximal tubular cell balooning and vacuolar degeneration in a pt with acute renal failure who has **Ca2+ oxalate crystals (buzz word for this!)** has ATN bc of why? WHAT **PT** **POPULATION** SHOULD YOU WATCH OUT FOR AS HAVING A TENDENCY TO BE ASSOCIATED WITH THIS
**ethylene glycol!!!!** **glycolic acid** is **toxic** to tubule cells AND **oxalic acid** precipates to **Ca2+ OXALATE stones**!!!!!!!!! watch out for antifreeze ingestion in **(homeless) ALCOHOLIC PTS** bc they ingest it in place of alcohol!
38
massive consumption of EGG whites (which contain lots of Avidin) can lead to what vit def?
BIOTIN!!!! b7!!!!!!! avidin binds b7 and prevents its reabsorption needed for carboxylase reactions which need ABCs ATP Biotin (b7) Co2
39
what are two diseases that are commonly associated as examples of the concept of **Mosaiciam**
**Turners syndrome (XO and XX in one person)** **McCune Albright Syndrome** [due to mutation affecting **G-protein signaling.** Presents with **unilateral café-au-lait spots**, **polyostotic brous dysplasia**,**precocious puberty**,**multiple endocrine abnormalities**. -----Lethal if mutation occurs **before** fertilization *(affecting all cells*), but **survivable in patients with mosaicism.**
40
what is the difference in how a a person with **HIV** is affected by **CANDIDA** versus a person who is **NEUTROPENIC**? [this is very important] side question: what other bug is known to affect neutropenic pts?
**T cells (Th)** protect **against SUPERFICIAL Candida** infections (oral, cutaneous, vulvovaginitis, **MUCOCUTANOUS**) so someone with **HIV** who has a **LOW T CELLS COUNT is affected with SUPERFICIAL CANDIDIASSIS** VERSUS **NEUTROPHILS PREVENT HEMATOGENOUS SPREAD OF CANDIDA** (dissemmmmminated infections: **CANDIDEMIA/endocarditis)** affected **NEUTROPENIC PTS or otherwise immunocompromised (CHEMOTHERAPY or INHERITED IMPAIRMENTS OF PHAGOCYTOSIS)** **aspergillus and psuedomonas bad for neutropenics too**
41
GIVE ALL THE MAIN EFFECTS OF CORTISOL ## Footnote THINK: Cortisol is a "**BIG FIB"** **WHAT AE CAN Exogenous corticosteroids** HAVE AS IT RELATES TO TWO SPECIFIC MICROBIAL INFECTIONS?
**INC B**lood pressure: * ƒ Upregulates α1-receptors on arterioles sensitivity to norepinephrine and epinephrine * ƒ***At high concentrations****,* can bind to mineralocorticoid (aldosterone) receptors **INC I**nsulin resistance (diabetogenic) **INC G**luconeogenesis, lipolysis, and proteolysis **DEC F**ibroblast activity (causes striae) InFLammatory and **I**mmune responses: * ƒ Inhibits production of leukotrienes and prostaglandins * ƒ Inhibits WBC adhesion **neutrophilia!!!! INCREASED NEUTROPHILS BC NOT MARGINATING!** * ƒ Blocks histamine release from mast cells * ƒ Reduces eosinophils * ƒ**B**locks IL-2 production **DEC B**one formation ( DEC osteoblast activity) **Exogenous corticosteroids** can cause reactivation of TB and candidiasis (blocks IL-2 production).
42
what are 3 RFs for **Neonatal respiratory distress syndrome?**
1. P**rematurity** 2. **Maternal diabetes** (due to fetal insulin) 3. **C-section delivery** (release of fetal glucocorticoids).
43
**Trypanosoma cruzi** which causes **Chagas disease**—**dilated cardiomyopathy** with apical atrophy, **megacolon,** megaesophagus; predominantly in **South America** **Unilateral periorbital swelling (Romaña sign)** characteristic of **acute stage** **is transmitted by WHAT and is treated HOW**
**Reduviid bug** (“kissing bug”) feces, deposited in a **painless bite** (much like a kiss) **Benznidazole** or **nifurtimox;** **Cruzi**ng in my **Benz**, with a **fur** coat on
44
is Sirolimus (Rapamycin) M TOR inhibitor NEPHROTOXIC? what drug is is SYNERGISTIC WITH
**NO!!!!!!!!** **hence why it can be synergistic with Cyclsporine which IS nephrotoxic**
45
abnormal ristocetin cofactor activity---no aggregation upon addition of Ristocetin....... WHAT DISEAES IS THIS DIAGNOSTIC FOR -how does this Ristocetin cofactor assay work? whats one weird-ish treatment for this disease but totally makes sense once you know the MOA?
**von Willebrand disease** **Platelet agglutination** caused by **ristocetin** can occur **only in the presence of von Willebrand factor multimers**, so if ristocetin is added to blood lacking the vwf, **the platelets will not clump.** The **antibiotic ristocet**in causes von Willebrand factor **to bind the platelet receptor glycoprotein Ib (GpIb),** so when ristocetin is added to normal blood, it causes agglutination. **Treatment: desmopressin**, *which releases vWF stored in endothelium.*
46
alcoholic takes ***_acetaminophen_*** **now he is jaundice** **has inc PT** **and real high AST** whats the pathogenesis too much NADH? not enough glutathione?
**Overdose produces hepatic necrosis;** ***_acetaminophen metabolite (NAPQI) depletes glutathione_*** ***---\>*** and forms **toxic tissue byproducts** in liver. **N-acetylcysteine** is **antidote**—*_regenerates glutathione._*
47
Minoxidil what would be the effect of placing Minoxidil in a solution of just saline and THEN adding a piece of vascular Sm to it?
DIRECT vasodilation Minoxidil is a **Direct** arteriolar vasodilator. uses for: -Androgenetic alopecia severe refractory hypertension. note that the fact that norepi or epi but just saline were added to the solution IMPLIES the the effect of this drug MUST be a direct effect on vasc sm NOT one mediated IN THE PRESENCE of norep or epi which is why adding something like Phentolamine would NOT cause the same vasodilatory effect even though we think of an alpha antag as opposing vasocontstriction and hence vasodilating. but it does it in the presence of Norepi. (thats the catch)
48
causes ORAL ULCERRRRR can survive INTRACELLULARLY prediliction for mononuclear phagocyte system so can lead to HEPATOSPLENOMEGALY cannot be transmitted person to person unlike to TB but like TB can form granulomas its smaller than an RBC its a mold in the cold and a yeast in the heat bird or bat droppings!!!! its HIDES in macrophages can cause pnm in a health chap but disseminates in IC people what organism is this
HISTOPLASMOSIS
49
what are the labs values like for a pt who is pregnant or taking OCPs? give what the levels of **Transferrin (TIBC)** and **% Transferrin Saturation** would be and why
**(inc Transferrin; dec % sat Transferrin)** **Estrogen** (as is seen **in inc amt** in both **Pregnancy & OCP** use)---\> **upregulation of TRANSFERRIN SYNTHESIS in the liver** this, in turn, alters the ratio that makes up % Transferrin Saturation): **Fe2+: TIBC** same as: Fe2+/TIBC in such a way that bc the liver is making MORE TIBC and the amt of Fe2+ has not changed or is even DEC in pregnancy bc of INC demand, the numerator gets smaller so the **%saturation DECREASES**
50
On inspirtation, is Jug Venous pressure supposed to normally increase or decrease? what is it called when this normal way doesnt happen?
**JVP** is supposed to **DECREASE** on **inspiration** **Kussamaul sign:** JVP increases on inspiration instead. seen in: Constrictive pericarditis Restrictive CM RA or RV tumors
51
which disease is being described: can present as **severe hemorrhagic diathesis** due to **vitamin K deficiency** (which creates a **HYPOcoagulable state**) and bleeding. is an unusual susceptibility to bleed (hemorrhage) mostly due to **hypocoagulability**, in turn caused by a coagulopathy (**a defect in the system of coagulation**). Several types are distinguished, ranging from mild to lethal.
**Celiac sprue** **(due to malabsortion)**
52
young child *not vacinnated* paroxysm of *machine gun-like coughing* and forced expiratory grunt INC LEUKOCYTES WITH *_LYMPHOCYTE PREDOMINANCE_* the culprit of this condition has "defective Neutrophil chemotaxis and oxidative metabilism due to **increased activity of ADENYLYL CYCLASE (cAMP)** **whats the orgnaism, describe it, what stain, name the clinical disease and whats the MOA behind this Increased cAMP?**
B. pertussis grame -ve cocobacillus stain = Bordet Gengou medium "WHOOPING COUGH" PERTUSSIS EXOTOXIN (ab toxin) **Overactivates adenylate cyclase (cAMP**) by **disabling Gi,** ***impairing phagocytosis to permit survival of microbe.***
53
what lung cancer is this ## Footnote grows along **alveolar septa---\> apparent “thickening” of alveolar walls**. Tall, columnar cells containing **mucus.**
its a subtype of adenocarcinoma IN SITU !!!!! called: Bronchioloalveolar subtype mucin +ve! Just like adenocarcinoma!
54
what in the world is so unique about **Trypanosoma brucei?** what is a big diff btw **Trypanosoma brucei & Trypanosoma cruzi**
famously causes **recurring fever** (due to antigenic variation)!!!!!!! changes its **VARIANT SURFACE GLYCOPROTEINs** every time host mounts and immune response!!!! **Trypanosoma brucei = Tsetse fly= a PAINFUL bite** **Trypanosoma cruzi = Reduviid bug (“kissing bug”)** feces**,** deposited in **a PAINLESS bite** (much like a kiss)
55
Lymphocytes are derived from
Lymphocytes of **mesenchymal origin.**
56
what vit deficiency can **OCPs** lead to clasically?
**Vit b6!!! (PLP)** along with INH
57
**Aldesleukin (IL-2)** is used to treat what two conditions mainly?
Renal cell carcinoma metastatic melanoma
58
59
which lung cancer can secrete b-hcg?
large cell lung ca Highly anaplastic undifferentiated tumor; poor prognosis. Less responsive to chemotherapy; removed surgically. **peripheral location**
60
Homer-Wright rosettes characteristic of:
**neuroblastoma** and **medulloblastoma.**
61
whats the AE of having LOW Mg2+?
Tetany, torsades de pointes, hypokalemia
62
**anti-Jo-1 abs** are **AKA**? what disease has these? whats it like on histo? CD4 OR 8 + T CELLS? WHATS THE MAIN MANIFESTATION? what other antibody is associated with it?
**anti-histidyl trna synthetase abs!**!!! **POLYMYOSITIS** **ENDOMYSIAL (vs PERImysial inflamm in Dermatomyositis)** INFLAMMATION WIHT **CD8+ T** CELLS **PROGRESSIVE/INSIDIOUS PROXIMAL MUSCLE WEAKNESS** *_"TROUBLE GOING UP STAIRS, GETTING OUT OF CHAIR"_* *_ANA ab_* *_ANTI-SRP ab_* *_ANTI-Mi-2 ab_* *_Increased CK and aldolase in serum_*
63
**segemental demyelination** of **peripheral nerves** with **ENDONEURAL INFLAMMATORY INFILTRATE** (with lymps and macs) is CHARACTERISTIC OF WHAT
**GUILLAIN BARRE!** (aka: acute **inflammatory demyelinating** polyradiculopathy)
64
herniation of gallbladder mucosa into the muscular wall is histologically called what and is associated with what type of Gall bladder disorder?
**CHRONIC CHOLECYSTITIS** **(Rokitansky-Aschotf sinus,** Chronic inflammation of the gallbladder Due to chemical irritation from longstanding cholelithiasis, with or without superimposed bouts of acute cholecystitis Characterized by herniation of gallbladder mucosa into the muscular wall **(Rokitansky-Aschotf sinus,** Fig. 11.3A) Presents with vague right upper quadrant pain, especially after eating Porcelain gallbladder is a late complication (Fig. 11.3B). 1. Shrunken, hard gallbladder due to chronic inflammation, fibrosis, and dystrophic calcification
65
AE of heparin? one REALLY IMPORTANT ONE
**Bleeding, thrombocytopenia (HIT),** **osteoporosis** drug-drug interactions. (NOTE: **enoxaparin, dalteparin:** LMWH does **NOT** cause osteoporosis), **Heparin-induced thrombocytopenia (HIT)—**development of **IgG antibodies** against **heparin- bound platelet factor 4 (PF4)** *BC (this _complex_ acts as a* **hapten) THAT ACTIVATES our immune system to form those antibodies** **Antibody-heparin-PF4 complex (now all three things are together forming a complex which** THEN **activates platelets--\>** thrombosis and thrombocytopenia (due to depletion). **For rapid reversal (antidote),** use **protamine sulfate** (positively charged molecule that *binds negatively charged heparin).* * note: PF-4 is a chemokine that is released from alpha-granules of activated platelets during platelet aggregation, and promotes blood coagulation by moderating the effects of heparin-like molecules* * replace hep with* * direct thrombin inhibitors (argatroban,* * bivalirudin, dabigatran)*
66
name 4 toxins that are taken up via retrograde axonal transport
**polio** **rabies** **herpes** **tetanus toxins** are (except herpes which is taken up via sensory fibers) take up via retrograde trasports via axons that **innervate skeletal muscle**
67
how much of the tensile strength will be regained after 3 months time in a scar?
**70–80%** of tensile strength regained at 3 months; little additional tensile strength will be regained afterward.
68
what interleukin is used to treat **Thrombocytopenia**? number and pharm name
**Oprelvekin (IL-11)**
69
**Restrictive lung diseases ƒcan be due to Interstitial lung diseases or** **Poor breathing mechanics, for example due to:** **1. Poor muscular effort: LIST 3 EXAMPLES** **ƒ 2. Poor structural apparatus: LIST 2 EXAMPLES** **what is the AA gradient in these?**
these are due to **Poor breathing mechanics, not an interstitial lung issue so the DIFFUSING CAPACITY is UNAFFECTED and therefore--\>** **n*_ormal A-a gradient):_*** **the issue is *Extrapulmonary, _peripheral hypoventilation (normal Aa with Hypoventilation, we know this)_*** **ƒ Poor muscular effort**—polio, myasthenia gravis, Guillain-Barré syndrome **ƒ Poor muscular effort**—scoliosis, morbid obesity
70
anti-smooth muscle abs seen in
**autoimmune hepatitis** middle aged woman with sxs of chronic hep
71
List all the CYP450 **INDUCERS** **SCR**(a)**PP NC BIGG**
St. John’s wort Chronic alcohol use Rifampin (a) Phenytoin Phenobarbital Nevirapine (NNRTI) Carbamazepine Griseofulvin Ginseng &
72
73
which vitamin can prevent morbidity and mortality from MEASLES (rubeola) especially in kids? only ONE single association here.
**VIT A!!!!!!!!!**
74
whats the relationship betwen **cross sectional area and resistance to flow**? where is resistance to flow the LEAST in the respiratory tree? and why????
**Resistanace=P/Area x Velocity** note: also the relationship btw Cross sectional area and Velocity of flow are INVERSE. inc A (cross sectional area)--\> DEC in Velocity of flow. (look at how flow velocity is different in capillaries vs, venules etc (KNOW THIS CONCEPT)-there are graphs showing this so if you increase cross sectional area you DECREASE resistance to flow as is seen in the TERMINAL bronchioles where resistance to flow is the LEAST of anywhere in the Respiratory tree and this is bc even though the diameters of the term are small there are many (inc cross sectional area) lined up in **PARALLEL** so this decreases the resistnace! (**highest resistnace** is seen in **MEDIUM** sized bronchii like: segmental and lobar bronchi)
75
**STERILE PYuria** and **NEGATIVE urine cultures** suggests what **UGI** infection
neisseria or chlamydia **URETHRITIS!** sterile pyuria is wbcs in urine "without bacteria"---but this is bc neiss and chlam are not seen on a gram culture!
76
systemic mastocytosis what the hell is that
77
whats the urachus? if a remnant of the urachus remains, what cancer can this cause?
In the 3rd week the yolk sac forms the--\> allantois, which extends into urogenital sinus.---\> Allantois becomes the---\> urachus (**a duct between fetal bladder and umbilicus)** **yolk sac---\> allontois---\> urachus** ***ADENOCARCINOMA OF THE BLADDDDERRR!!!!!!!*** **1. a urachal remnant-eg: urachal cyst (**fluid-filled cavity lined with uroepithelium, between umbilicus and bladder) **-(tumor develops at the DOME of the bladder),**
78
LIST 3 drugs with **Zero-order elimination** [**Capacity**-limited elimination] not flow dependent like first order elim
Examples of drugs— **P**henytoin, **E**thanol, and **A**spirin (at high or toxic concentrations). Rate of elimination is constant regardless of Cp (ie, constant **AMOUNT** of drug eliminated per unit time).
79
Calcium phosphate needs inc or dec PH to form? how about calcium oxalate?
Calcium phosphate: **INC pH** **hypocitraturia (with DEC ph)--\> calcium oxalate or phosphate** **radioopaque can see on xray**
80
which local anesthetics (in the Amide category) has an AE of: **severe cardiovascular toxicity**
**Bupivacaine** note: Esters just have **1 "i"** in their name, ulike the amides. but **both end in: -caine**
81
**Red “currant jelly” stools means what in an adult vs an infant?**
**Acute mesenteric ischemia** (adults) **intussusception** (children)
82
whats the **most common presentation** of an eldersly person with atherosclerosis who has **ATHERO-EMBOLIC disease** after an **invasive procedure**, like an cardiac catheritizatoin)
**ATHERO-EMBOLIC DISEASE** (THINK ABOUT THE NAME) -during that invasive procedure like aortic catheritizatoin, **CHOLESTEROL** containing debris can **DISLODGE** and travel to smaller vessels where they block the area and cause ischemia to that tissue which presents a few days to weeks post surgery as: MOST COMMONLY: **acute kidney injury!!!!** (oliguria and azotemia)----THE PATIENT **WOULD NOT HAVE A FRANK INFARCTION WITH HEMATURIA AND FLANK PAIN** AND THE WHOLE SHABANG BC CHOLESTEROL EMBOLI ARE WITTLE. ON HISTO (if in the kidney) you could see **needle shaped cholesterol clefts** that partially or completely obstruct the arcuate or intralobular renal arteries other organs that can be involved are: GI, CNS (like retina vessels)
83
what makes up the RMP of a muscle cell whose RMP is -70?
High K+ conductance (efflux) and some Na+ conductance
84
**"no platelet clumping"** seen on blood smear. what diseae? what are the labs
**Glanzmann thrombasthenia** **DEC** GpIIb/IIIa--\> defect in platelet-to-platelet aggregation. **Labs**: blood smear shows no platelet clumping + INC BT
85
the are characteristic labs of what disease ## Footnote Findings: CSF protein with normal WBC cell count (albuminocytologic dissociation). protein may cause papilledema.
Guillain-Barré syndrome.
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**IV drug users** who get infected with **candida** can get WHAT manifestation?
**endocarditis!!!!!** from candida! in **(IV drug users)**
87
what is the 1. pathogenesis 2. lab values seen (are these same lab values shared with any other disease?) 3. treatment for **Hemochromatosis**
1. pathogenesis: Recessive mutations in HFE gene (C282Y chromosome 6) **abnormal iron sensing and INCREASED intestinal absorption** **2. LABS:** (INC ferritin, INC iron, DEC TIBC--\>INC transferrin saturation)---**SAME as** *SIDEROBLASTIC ANEMIA* 3. TX: repeated phlebotomy, chelation with **deferasirox, deferoxamine, oral deferiprone.**
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what genetic principle is this an example of? **Different mutations** in the **same locus** produce **the same phenotype.** **give 2 relevant clinical examples of this.**
**Allelic heterogeneity** * **b or a-Thalassemia** * **G6PD deficiency (there are over 400 *diff* mutations on the *same* disease-causing locus in G6PD that are knows to cause this deficiency)**
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if a **Diabetic** patient taking **diurects** (volume depleted) shows **albuminuria** **so you decide to give her an ACE-I to treat her "early diabetic nephropathy"** **and then the next day the pt returns to you and says they are lightheaded and almost fainted,** **why is this happening?**
**first dose HYPOtension (AE)** can occur if someone is volume depleted and also takes an ACE-I since they block vasoconstriction
90
Degenerative disorder of CNS associated with **Lewy bodies** (composed of **α-synuclein—intracellular eosinophilic inclusions** **brain slice on gross looks like...**
the ***_pathologic hallmark of Parkinson's disease_*** remains **synuclein-filled Lewy bodies in the nigrostriatal system.** eg: 67-year-old man with 1 year progressive difficulty writing and walking. Pt is stooped and talks slowly. PE shows **bland facial expression**, fine **resting tremor in both hands, no tremor when moves**, walks with difficulty **starting and stopping, cogwheel rigidity.** Brain tissue histology shown as well as gross cross sections of midbrain both normal and diseased.
91
whats the **MOA** of **(sodium) NITROPRUSSIDE**? whats the **main AE?** and what can you give as an antidote? **WHATS SITUATION** IS IT USED IN as the **DOC**?
MOA is that **NITROPURSSIDE DIIIIRREEECCCTTTLLLLYYYYYYYYY** releases **NO** and causes and **increase in cGMP** leading to **VASO and VENO dilation** and is the: **DOC for HYPERTENSIVE 911's** bc of its **SHORT t1/2** **main AE= CN toxicity (cn is a metabolite)** **antidote: SODIUM THIOSULFATE** helps make thiocyanate which **can be eliminated:)**
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what are the **TWO DIFFERENT mechanisms** by which THYROID HORMONE INC SNS ACTIVITY AND BASAL METABOLIC RATE?
**INC SNS activity:** via **stim of β1 receptors** in heart = CO, HR, SV, contractility **INC basal metabolic rate** via **INC** synthesis of: **Na+/K+-ATPase!!!!!!!!!!!!!!!--\>** activity INC O2 consumption, RR, body temperature!!!!!!!!
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what two lab values are INC in Wiskott-Aldrich syndrome Mutation in **WAS gene;** **T cells unable to reorganize actin cytoskeleton.** X-linked recessive.
INC **IgA!!!!** **IgE!!!!!** W**A**T**E**R
94
what are the 3 main (general) RFs contributing to the general formation of Gall bladder stones (cholelithiasis)
INC cholesterol and/or bilirubin DEC bile salts, gallbladder stasis all cause stones
95
a PRIMARY CNS lymphoma. whats is the FIRST AND MAIN ASSOCIATION you should think of ?
HIV/AIDS!!!!!!!!!!!!!!!!!!! **Considered an AIDS-defining illness.** Variable presentation: confusion, memory loss, seizures. Mass lesion(s) on MRI, needs to be distinguished from toxoplasmosis via CSF analysis or other lab tests.
96
what are the three ways one could get **ADENO CA of the *bladder*** ***weird, right?*** note: these all have to be things that either already have glandular columnar epi or cause it to form (bc normally bladder is transitional epi)
Malignant proliferation of glands, usually involving bladder Arises from 1. **a urachal remnant-eg: urachal cyst-**(tumor develops at the ***_DOME_*** of the bladder), 2. **cystitis glandularis** **(causes columnar metaplasia!)** 3. bladder **exstrophy** (congenital failure to form the caudal portion of the anterior abdominal and bladder walls)...bc bladder exposed to outside world and can cause is to get cancer and change epi
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Endometriosis. what are some defining features that make it DIFF from leiomyoma Non-neoplastic endometrial glands/stroma outside endometrial cavity F . Can be found anywhere; most common sites are ovary (frequently bilateral), pelvis, peritoneum. In ovary, appears as endometrioma (blood- lled “chocolate cyst”). May be due to retrograde ow, metaplastic transformation of multipotent cells, transportation of endometrial tissue via lymphatic system. Characterized by ***_cyclic pelvic pain,_*** bleeding, dysmenorrhea, dyspareunia, dyschezia **(pain with defecation)**, infertility; normal-sized uterus. **NSAIDs, OCPs, progestins, GnRH agonists, danazol, laparoscopic removal.**
**Symptoms can vary depending on location of implants.** **ITS CYCLICAL and IT HURTS!!!** **leiomyomacause===\> abnormal uterine bleeding, or result in miscarriage. Severe bleeding may lead to iron de ciency anemia.** **extra manifestations of endometriosis!** * Large intestine: **Pain during defecation,** abdominal bloating, or ***_rectal bleeding during menses!!!!!!!!!!!!!_*** * Bladder: Dysuria, hematuria, suprapubic pain (particularly during urination), or a combination * Ovaries: Formation of an endometrioma (a 2- to 10-cm cystic mass localized to an ovary), which occasionally ruptures or leaks, causing acute abdominal pain and peritoneal signs * Adnexal structures: Formation of adnexal adhesions, resulting in a pelvic mass • Extrapelvic structures: Vague abdominal pain (sometimes)
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HEMATOCHEZIA IS MOST COMMONLY ASSOCIATED WITH WHAT TWO CAUSES OF GI BLEEDING
**DIVERTICULOSIS (left sided!)** **ANGIODYSPLASIA** (**right sided!** **-**-cecum, terminal ileum, ascending colon **BOTH COMMON IN *_PPL GREATER THAN 60_*** **_also ischemia colitis (colonic ischemia) which unlike acute (due to embolism in SMA) and chronic mesenteric ischemia (due to atherosclerosis of celiac, sma, or ima), is NOT due to obstruction._** **_\*while the mesenteric ischemias present with lots of pain_** **_Colonic ischemia is due to HYPOPERFUSION eg: Hypotension; presents with crampy pain_**
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pyloric stenosis is associated with **WHAT class of antibiotics**? billious or non billious vomit? whats the clinical buzz word? whats the time frame relative to birth?
**MACROLIDES!!!!!!** **NON billious** (unike duodenal atresia) **"palpable olive mass"** in the epigastric region **2-6 WEEKS post birth** (inlike duodenal atresia seen 1-2 DAYS) side note on AE of macrolides: **MACRO:** Gastrointestinal **M**otility issues\*\*\*\*\*\*\*\*\*\*\*\*\*\* **A**rrhythmia caused by prolonged QT interval acute **C**holestatic hepatitis, **R**ash, e**O**sinophilia
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**phenytoin, sulfonamides, methotrexate, trimethoprim** all have in common that they can cause what type of vit def? what are the **lab**s in this def? **homocysteine & methylmalonic acid levels** specifically. **WHY?** **draw out the pathway please**
**folate** vit b9 **INC** homocysteine **normal** methylmalonic acid levels. read the description to understand also refer to FA for a simpler version of the pathway
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new holosystolic murmur Immune mediated Type II hypersensiticity (due to molecular mimicry) Interstitial myocardial granulomas (which have another name) 10 year old boy with: fever, poor appetite, hypotension, tachycardia (are sxs of what characteristic finding in this disease process) what disease
## Footnote **\*Rheumatic fever** **\***Immune mediated (type II hypersensitivity); not a direct effect of bacteria. Antibodies to M protein cross-react with self antigens **(molecular mimicry).** \*Interstitial myocardial granulomas aka: **ASCHOFF BODIES** \*Murmur bc an Early lesion is **mitral valve regurgitation** **boy had signs and sxs of MYOCARDITIS**
102
**Amphotericin B & sodium stibogluconate** treat a condition that is diagnosed by seeing **Macrophages containing amastigotes** what condition is this
**Visceral leishmaniasis** (kala-azar)—**spiking fever**s, hepatosplenomegaly, pancytopenia **Cutaneous leishmaniasis**—skin ulcers **Sandfly trasmits it!!!** **its a protozoal infection**
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resting membrane potential for a cells is determined by conductance of....
high K+ conductance (some) Na+ conductance
104
what cytokine is inc in Multiple Myeloma? what type of Amyloidosis is seen?
**IL-6** *It has been demonstrated to be involved in the proliferation of plasmablastic cells in bone marrow and in the differentiation of these cells into mature plasma cells.* **AL (primary) 1º AMYLOIDOSIS!!!!** **Due to deposition of proteins from Ig Light chains.** Can occur as a **plasma cell disorder or associated with multiple myeloma**. Often affects multiple organ systems, including renal (nephrotic syndrome), cardiac (restrictive cardiomyopathy, arrhythmia), hematologic (easy bruising, splenomegaly), GI (hepatomegaly), and neurologic (neuropathy).
105
compare type 1 and 2 diabetes in all the ways you can
106
Rib notching (inferior surface, on x-ray) is seen in what condition commonly how come?
**Coarctation of the aorta** Aortic narrowing near insertion of ductus arteriosus (“juxtaductal”). Associated with **bicuspid aortic valve, other heart defects, and Turner syndrome**. Hypertension in upper extremities and weak, delayed pulse in lower extremities **(brachial-femoral delay).** W**ith age, intercostal arteries enlarge due to collateral circulation; arteries erode ribs notched appearance on CXR.** Complications include HF, risk of **cerebral hemorrhage (berry aneurysms), aortic rupture**, and possible endocarditis.
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pt has a disease that is classicaly treated with: **metronidazole or oral vancomycin**. For recurrent cases, consider repeating prior regimen, **fidaxomicin,** or fecal microbiota transplant. caused by: an **anaroebic**, **spore forming gram +ve bacteria** Diagnosed by detecting one or both toxins in stool by **PCR.** Presents with: **watery diarrhea** most commonly whats the etiology of this disease usually? whats the disease? what are the two toxins and their mechanism?
**Toxin A**, ***_enterotoxin_***, binds to the brush border of the gut. **Toxin B**, ***_cytotoxin,_*** causes cytoskeletal disruption via **actin depolymerization--\>** diarrhea--\> **pseudomembranous colitis!!!!!** **due to antibiotics**
108
any time a CoA is seen in a biochem rxn WHAT vitamin is also needed
Vitamin B5!!!! (pantothenic acid!) eg: FA oxidation (FaCoA SYNTHASE and CPT (an acyl transferase) need it!!! FA--\> fatty acyl co A
109
A **staccato cough** is consistent with what..
with a **viral** or **atypical pneumonia (eg: *NEONATAL PNM CAUSED BY CHLAMIDYA -----here you also eosinophilia)***
110
what two carcinogens can lead to ANGIOSARCOMA of liver
ARENSIC VINYL CHLORIDE
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Hirstuism + SECONDARY amenorrhea
PCOD
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**Thrombosis and (in pregnancy) fetal demise** associated with various autoimmune antibodies directed against one or more phospholipid-binding proteins **(eg, β2-glycoprotein I, prothrombin, annexin).** These proteins normally bind to phospholipidmembrane constituents and protect them from excessive coagulation activation. The autoantibodies displace the protective proteins and, thus, produce procoagulant endothelial cell surfaces and cause **arterial or venous thromboses.** whats the diag?
Antiphospholipid Antibody Syndrome (Anti-Cardiolipin Antibodies; Lupus Anticoagulant)
113
in someone with COPD exacerbation, if you give HIGH flow O2 shortly after they become increasinly lethargic and confused, what is this due to? ROS production or dead space ventilation
inc physiological dead space ventilation leading to v/q mismatch bc **poorly ventilated areas are perfused!** **see attached photo for the three reasons you get** ***OXYGEN INDUCED HYPERCAPNEA IN COPD!!!!*** note: **ROS prod would present differently** *like pleuritic chest pain, cough, dyspnea*, **WITHIN 24 HOURS OF BREATHING PURE 02**
114
Most common ectopic thyroid tissue site is the (lingual thyroid) located where... Thyroglossal duct cyst presents as mass where....
Most common **ectopic thyroid tissue** site is the tongue **(lingual thyroid) *@ base of tongue*!** note: Removal may result in hypothyroidism if it is the only thyroid tissue present. **Thyroglossal duct cyst:** presents as an ***anterior midline neck mass*** that **moves with swallowing** or protrusion of the tongue note: (vs persistent cervical sinus leading to branchial cleft cyst in lateral neck).
115
name the **6** main post **translational** modifications that consist of **Covalent** alterations **"m-a-p h-u-g"**
methylation, acetylation Phosphorylation, hydroxylation, ubiquitination. glycosylation,
116
**bivalirudin** **argatroban** **dabigatran** are....
**DIRECT THROMBIN INHIBITORS!** **if you're bleeding too much** Since theres no specifoc reversal agent. Can attempt to use activated prothrombin complex concentrates (PCC) and/or fibrinolytics (eg, tranexamic acid**).**
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PBG deaminase def--\> Uropor Decarboxylase def---\>
**PBG *Deaminase*** def--\> **Acute intermittent porphyria** note: this is the **2nd enzyme** used in the **CYtoplasm** during heme syn (the first is ALAD) **Uropor *Decarboxylase*** def---\> **Porphyria cutanea tarda** **note:** this is the **4th enzyme** used in the **CYtoplasm** during heme synth
118
DOES THE REID INDEX FOR CHRONIC BRONCHIITS INCLUDE THE CARTILAGE LAYER?
NOOOO!!! JUST THE PARTS **BTW** THE RESP EPITHELUM AND CARTILAGE
119
Fever + Absolute neutrophil count of\_\_\_\_\_\_\_\_\_\_is called Febrile Neutropenia and it puts pts at high risk of being infected with what 3 types of bug classes?
Absolute neutrophil count of **\<500mm** **FUNGI and VIRUSES & bacteria (pseudomonas)** **eg: Aspergillus Fumigatus--\> Invasive Pulmonary Aspergilosis (pneumonia)**
120
does neisseria meningitis grow on blood agar? how is it transmitted?
NOOOOO needs chocalate agar! in 5% CO2 atmosphere RESPIRATORY DROPLETS
121
since both **Riedel thyroiditis** *_AND_* **anaplastic carcinoma can extend to local structures** (eg, trachea, esophagus) **HOW DO YOU TELL THE DIFF** ON AN EXAM (ASIDE FROM HISTO BC Riedel thyroiditis IS HARD AS A ROCK BC ITS ALLLLL FIBROSED. WHAT OTHER CLUE CAN YOU USE?
AGEEEEE!!!!!!!!!! **Riedel thyroiditis *_is young female!!!!_*** *_AND_* **anaplastic carcinoma is ELDER!!!!!!!!!!!** **dont you frickin fall for this trick on the test day.**
122
**timeline is the key** diff btw **PTSD** and acute stress disorder **whats the diff**
**GREATER (\>1month)** for **ptsd** **3d-1** month and no longer! for **brief psychotic disorder**
123
Angiography revealed multiple or anomalous renal arteries going to each kidney are associated with what renal congenital defect?
**horseshoe kidney!** due to the halted ascent, renal vascular anomalies are common: usually multiple renal arteries arise from the distal aorta or iliac arteries; this is important when these patients undergo any procedure, particularly a renal angiogram not multiple ureters,
124
**"AZUROPHILIC"** + immature (blast cells) on blood smear SHOULD IMMEDIATELY MAKE YOU THINK OF WHAT
FREAKIN **AML!!!!!!!!!!** **AZUROPHILIC RODSSSSSSSSSSS** PLUS IMMATURE CELLS HTEY ARE **MPO +VE** INCREASED NUMER OF **MYELOBLASTS**
125
Lithium and amiodarone both require what kind of test bf starting meds
TSH
126
what cell in the stomach is this?
parietal cells light pink with blue nucleaus in the middle looks like a fried egg This slide shows several parietal cells. Parietal cells have a characteristic “fried-egg” appearance, with a basophilic, peripherally located nucleus and a rather eosinophilic cytoplasm. **These cells secrete HCl and intrinsic factor.** Under the electron microscope, the parietal cell appears pyramidal, with a central nucleus and many mitochondria. Why may mitochondria be so numerous in parietal cells?
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Thyroid tissue is derived from**....** Parafollicular cells (aka, C cells) of the thyroid are derived from**...**
Thyroid tissue is derived from **endoderm.** Parafollicular cells (aka, C cells) of the thyroid are derived from **neural crest.** **derived from the 4th pouch** **eg: medullary Ca of the thryroid is a**
128
autosomal recessive, profound bilateral sensorineural deafness + LONG QT interval + congenital what disease?
Jervell and Lange-Nielsen syndrome
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45-year-old man with hypertension not compliant with medications. bp 160/100. Cardiac exam shows apical impulse displaced laterally, loud S2 and S4 gallop. Echo shows thickening of left ventricular wall. Mechanism of change in cardiac muscle? The **levels of the following are inc or decreased**? **Transcription factor c-Jun** is... **beta-myosin heave chain** is.. **Endothelin** is... what even is endothelin? side q: what med inhibits endothelin and what is this med used to treat?
**c-Jun** is a protein that in humans is encoded by the JUN gene. **c-Jun** in combination with **c-Fos,** forms the **AP-1** early response transcription factor. NOTE! **C-JUN and C-FOS** are ***_nuclear proto-oncogenes and are induced in patients with hypertrophic cardiomyopathy,_*** **Overexpression of c-jun i**n cells results in **decreased level of p53 and p21**, and exhibits ***_accelerated cell proliferation (hence the hypertrophic CM)._***
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**rugal hypertrophy (lots of mucus prod)** dec acid secretion loss of protein (albumin)--\> edema stomach looks like brain gyri
**Menetrier!!!!!**
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is smoking a RF for mesothelioma?
NOOOO!!!!!!! but asbestos is!
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QID 79 GREAT PATHOGENESIS OF ACUTE CALCULOUS CHOLECYSITIS
133
which worm worm infection gives you granulomas!
schistosoma!!!!!!!! tx: DOC prazi
134
List 3 meds (antihypertensives) that increase Plasma Renin activity (specific names too)
ANTIHYPERTENSIVE MEDS 1. **Diuretics** (HYDROCHLOROTHIZIDE) 2. **ACE inhibitors** (Captopril, enalapril, lisinopril, ramipril ) 3. **Antiotensin II receptor blockers** (Valsartan. Losartan, candesartan,)
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can a **Tzank smear** where epithelial cells are scraped from an ulcer base and stained which show the presence of multinucleated giant cells suggest Herpes be able to tell you whether the infection is **HSV vs Varicella Zoster**?
**NO!** therefore, **PCR** has largely replaced it.
136
how do you mainly diff btw lambert eaton and MG?
Proximal muscle weakness (cant go up stairs, get up from chair) that improves with use **Autonomic symptoms _are huge_ and not seen in MG** like: **DRY MOUTH** **IMPOTENCE** also see **HYPO or AREFLEXIA!!!!!!**
137
whats the "wheel shaped virus and all its defining characteristics" here is the nbme q on it 2-month-old female with T 102F, vomiting, diarrhea, dehydration. Exam of stool shows viral particles with **wheel-like shape.** Properties of virus? Type of nucleic acid/envelop/capsid symmetry
- Double-stranded RNA (weird) segmented /no envelope-NAKED /icosahedral bc +VE sense! REO the weirdo causes ROTAvirus (also causes Colorado tick fever) all negative sense are HELICAL
138
which gram POSITIVE bug has an ENDOtoxin? not common. are exo or endo toxins ACTIVELY secreted?
eXO toxins are actively secreted ENDO toxins are not Listeria monocytogenes listerolysin O and it helps the organism evade phagocytosis
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* **New-onset hypertension** with either proteinuria or end-organ dysfunction **after** 20th week of gestation (\< 20 weeks suggests molar pregnancy). * **whats the pathogenesis of this condition?** * Incidence in patients with pre-existing hypertension, diabetes, chronic renal disease, autoimmune disorders. * **Complications:** placental abruption, coagulopathy, renal failure, uteroplacental insufficiency, eclampsia.
Pre eclampsia May proceed to **eclampsia** (*pre-eclamp + maternal seizures*) and/or **HELLP** syndrome. **Caused by abnormal placental spiral arteries** **endothelial dysfunction, vasoconstriction &** **ischemia.** **Treatment: antihypertensives, IV magnesium sulfate (to prevent seizure); de nitive is delivery of fetus.**
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* lipohylainosis + microatheroma --\> small vessel occulusion * thalamic stroke (VENTRAL POSTERIOR THALAMUS) with **PURE SENSORY LOSS** eg: DCML and ALS (anterolat/aka: spinothalamci) messed up * history or uncontrolled HTN and DM WHAT KIND OF INFARCT DO YOU NEED TO BE THINKING OF?
LACUNAR INFARCT they are result of small vessel occlusion due to lipohyalinosis and microatheroma in pentrating vessels of DEEP brain structures
141
Malignant mesenchymal proliferation of immature skeletal muscle; rare **Presents as bleeding and a grape-like mass protruding from the vagina or penis of a child (usually\< 5 yrs of age); also known as sarcoma botryoides** **Rhabdomyoblast, the characteristic cell, exhibits cytoplasmic cross-striations and** positive immunohistochemical staining is seen FOR....what two things what disease is this?
positive immunohistochemical staining for **desmin and myogenin.** **rhabdomysosarcoma**
142
DRY INSPIRATORY CRACKLES COUGH "PATCHY INTERSTITIALLLLLL INFILTRATION" DYSPNEA FEVER are an AE of which of the following drugs? - prednisone - sotalol - amiodarone - levothyroxine
**AMIODARONE!** i was describing characteristic features of inflamation and **FIBROSIS in the pulmonary intersitium!** will present with **PROGRESSIVE DYSPNEA & DRY COUGH!** AMIODARONE CAUSES **INTERSTITIAL PNEUMONITIS (pulmonary fibrosis!)!!!!!!!!**
143
whats a very common cause of: ***Neonatal* hemorrhage** with PT and aPTT but normal bleeding time what is done to prevent this whats the pathogenesis of this
**Vit K deficiency** Vit K is Synthesized by intestinal flora. (neonates have **sterile intestines** and are **unable to synthesize vitamin K**). \*\*\*Can also occur after **prolonged use of broad-spectrum antibiotic** Newborns, as mentioned above, fit into this category, **as their colons are frequently not adequately colonized in the first five to seven days of life** since Vit K is not in breast milk; neonates are given **vitamin K injection at birth to prevent hemorrhagic disease of the newborn.**
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what bug does this: ## Footnote Causes acute diarrhea or **pseudoappendicitis** (right lower abdominal pain due to mesenteric adenitis and/ or terminal ileitis).
Yersinia enterocolitica Gram ⊝ rod. Usually transmitted from pet feces (eg, puppies), contaminated milk, or pork.
145
whats the **diff** btw **ACUTE and CHRONIC** mesenteric ischemia
**Critical blockage** of intestinal blood flow (often **embolic occlusion of SMA**) *_small bowel necrosis_*--\> abdominal pain **out of proportion** to physical findings. ^^^[May see red “currant jelly” stools.] **“Intestinal angina”**: **atherosclerosis of celiac artery, SMA, or IMA--**\> *_intestinal hypoperfusion postprandial--\> epigastric pain--\> food aversion and weight loss._*
146
whats the difference between the pathogenesis btw Albinism and Vitiligo? match: **decreased TYROSINASE activity**--\> **decreased MELANIN** production (with **NORMAL** NUMBER OF **MELANOCYTES)** **autoimmune destruction of MELANOCYTES**--\> depigmentation (strongy associated with other autoimmune disorders such as: Graves, Autoimmune thyroiditis, Type 1 diab, pernicious anemia)
decreased Tyrosinase activity and decreased/absent MELANIN prod = **ALBINISM** autoimmune destruction of MELANOCYTES= **VITILIGO**
147
ae of CCBs both types
**verap, dili! Non-dihydropyridine:** cardiac depression, AV block, hyperprolactinemia, constipation. **the -dipines! Dihydropyridine:** *peripheral edema,* ushing, *dizziness, gingival hyperplasia.* *MOA below*
148
what is one pathogenic mechanism why gynecomastia is seen in cirrhosis?
decreased degradation of estrogen by the liver. degrad/metabolism of estrogen to its metabolites bf being urinated out occurs in large part by the CYP450 enzymes in the lIVER
149
a baby has **jejunal/ileal atresia.** how is the bowel affected? name 3 distinct features in order from most proximal bowel to most distal.
1. the proximal jejunum ends in a BLIND POUCH 2. followed by an are of absent bowel 3. KEY: terminal ileum distal to atresia spirals (like an APPLE CORE OR XMAS TREE) **around a**n ileocecel **bloodvessel** (aroudn a " thin vascular stalk)
150
which ligament contains the Portal triad? what are the contents of the portal triad? **Hepatoduodenal** **Falciform** **Gastrocolic** **Gastrohepatic**
**Hepatoduodenal** Portal triad: proper hepatic artery, portal vein, common bile duct
151
if a patient comes in (kid or adult) with mucosal bleeding, easy brusing & pettichiae **AND THEIR ONLY LAB ABNORMALITY IS A DECREASED PLATELET COUNT** -OF, for example, 9,000 **(NORMAL: 150,000-400,00)** WHAT ARE YOU THINKING OF **since they have an absolutely normal physical exam otherwise**.
**freakin ITP!!!!!** **autoimmune platelet destruction** by autoimmune platelet antibodies (IgG autoantibodies against plt **membrane glycoprotein GPIIb/IIIa)** in kids (eg: after viral infection or immunization) its self limited, in adults (most common in woman of child bearing age (or with SLE): can cause short lived thrombocytopenia in offspring since IgG can cross the placenta) its an insidious chronic course \*can see INC megakaryocytes on BM biopsy.
152
what **arthropathy is** **Hemochromatosis** associated with? affects knee alot
**Calcium pyrophosphate deposition disease** Deposition of calcium pyrophosphate crystals within the joint space. Occurs in patients \> 50 years old; both sexes affected equally. Usually idiopathic, sometimes associated with hemochromatosis, hyperparathyroidism, joint trauma. Pain and swelling with acute in ammation (pseudogout) and/or chronic degeneration (pseudo- osteoarthritis). Knee most commonly affected joint. Chondrocalcinosis (cartilage calci cation) on x-ray. Crystals are rhomboid and weakly ⊕ birefringent under polarized light (blue when parallel to light)
153
Anticentromere ab associated with
***Limited* scleroderma (**_C_**REST syndrome) shows** Anti**_C_**entromere abs! **C for C!!!**
154
what are the two cell surface markers **INHIBIT T cells** (and their response to tumors, for example). **list 2 and their ligands** antibodies against these CD markers are used to treat what type of cancer
**CTLA-4 (CD152)** on ACTIVATED T cells binds **CD80/86 (B7**) on its target (APC, tumor cells) MOA: competes with CD28 on t cells for binding to B7 on APCs how we are used to seeing (which is normally stimulatory) and CTLA4 has a higher affinity for B7 so it wins---\> no t cells--\> no tumor apoptosis--\> cancer. AND **PD-1** on ACTIVATED T cells binds **PD-1L** on its target (APC, tumor cells) NOTE:used to treat **ADVANCED MELANOMA**
155
A 25-year-old woman comes to the physician because of a 2-year history of intermittent, diffuse, cramping lower abdominal pain. The pain is usually associated with 2 to 6 days of loose, watery stools, and is typically relieved with defecation. Between these episodes, her stools are normal. Her vital signs are within normal limits. Physical examination shows no abnormalities. Laboratory studies, including complete blood count, metabolic panel, and thyroid function tests show no abnormalities. **A drug targeting which of the following mechanisms of action is most appropriate for this patient?** **a. inhibition of TNF-a** **b. accentuation of bile salt reabsorption** **c. Accentuates of μ-opioid myenteric plexus receptor​**
intermittent relieved with defecation is IBS for IBS diarrhea you can give - **LOPERAMIDE**: Accentuates of μ-opioid myenteric plexus receptor--\> dec Ach release and then DECREASE GI motility. note: Cholestyramine (now spelt colestyramine) has been given for many years for diarrhoea in patients with ileal resection (bc they cant absorb bile acids) so you give a bile acid binding resin in this case
156
what two things does cytokine IL-4 do? Hows about IL-5?
**IL-4** 1. promotes T helper cel differentiatoin into the **Th2 subset** 2. Stimulates B cell growth adn **isotype switch to IgE** **IL-5** 1. promotes growth and differentiation of **b cells and eosinophils** 2. Stimulates **isotype switching to IgA**
157
Synovial fuid **non-in ammatory** (WBC **\< 2000**/mm3). Involves DIP (Heberden nodes) PIP (Bouchard nodes) and 1st CMC **not MCP.** **OA or RA?**
OA
158
WHAT PULMONARY COMPLICATION IS ASSOCIATED WITH MARFANS?
SPONTANEOUS PNEUMOTHORAX FROM APICAL BLEBS
159
whats another way to say reverse transcriptase?
**RNA dep DNA pol** \*NOTE RETRO has a RNA dep DNA pol (reverse transcriptase) Naked nucleic acids of **⊝ strand ssRNA** and **dsRNA** viruses are **not infectious. need RNA dep RNA Pol (note these are all NEGATIVE)** **BUT** most **dsDNA** (except poxviruses and HBV) and **⊕ strand ssRNA** ( which is same as ≈ mRNA) viruses **are infectious.**
160
diff btw febrile non hemolytic and acute hemolytic transfusion rx?
**febrile non hemo** is due to host abs against donor **HLA** ags and **wbcs (just fever, chills, flushing and HA)** vs **hemolytic type** is due to **abo group i**ncompatibility or **host abs against foreign ags on donor RBCS (more severe)...HEMOGLOBINURIA (this is due to COMPLEMENT MEDIATED CELL LYSIS)**
161
macrophages containing golder cytoplasmic granules that turn dark blue with prussian blue stain are describing WHAT and WHAT is the pathogeneiss and most common cause of this finding in the LUNG THIS IS CRUCIAL TO KNOW!!!!
freakin **hemosiderin laden macrophages** MOST FREAKIN COMMONLY **CAUSED BY LEFT HEART FAILURE** LEADING TO INC HYDROSTATIC P IN THE CAPS==\> PULMONARY EDEMA. THE RISE IN PRESSURE OF THE BLOOD INTO THE LUNGS INC CAPILLARY PERMEABILITY AND **DISRUPTS THE CAPILLARY ENDOTHEIAL AND ALVEOLAR EPITHELIAL BLOOD GAS BARRIER!!!!** BLOOD LEAKS INTO ALVEOLI (EXTRAVASTION) AND THERE YOU HAVE IT **ALVEOLAR HEMMORAGE!!!!** **RBC ARE PHAGOCYTOSED BY MACS AND IRON FROM THE HB IS CONVERTED TO HEMOSIDERIN!!!**!
162
what autosomal recessive disease resulting from an ABNORMAL POST TRANSLATIONAL PROCESSING OF A TRANSMEMBRANE PROTEIN (WHERE ITS MISFOLDED AND NOT GLYCOSYLATED AND SO THE ER DIRECTS IT TO A PROTEOSOME FOR DEGRADATION)
CF
163
talk about: **Infections causing brain abscess (fill in the blank)** Most commonly **\_\_\_\_\_**and\_\_\_\_ If dental infection or extraction precedes abscess, **\_\_\_\_\_\_\_**commonly involved. * *Multiple abscesses** are usually from \_\_\_\_ * *Single lesions** from **\_\_\_\_\_\_\_** ----**otitis media and mastoiditis---\>what lobe in brain\_\_\_\_\_\_\_\_\_\_\_** **------sinusitis or dental infection----\> what lobe in brain\_\_\_\_\_\_\_\_** **Toxoplasma** is most commonly seen in\_\_\_\_\_\_\_\_
Most commonly **viridans streptococci** and **Staphylococcus aureus**. If dental infection or extraction precedes abscess, **oral anaerobes** commonly involved. **Multiple abscesses** are usually from **bacteremia; Single lesions** from **contiguous sites**: ----**otitis media and mastoiditis---\>** t**emporal lobe and cerebellum;** **------sinusitis or dental infection----\> frontal lobe.** **Toxoplasma** reactivation in **AIDS.**
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name that pneumoconiosis! **“Ivory white,” calcifed, supradiaphragmatic and *_pleural plaques._* pathognomonic of ....** **aerospace and manufacturing industries.** **Noncaseating granulomas** on histology in the lung, hilar lymph nodes, and systemic organs and therefore *occasionally responsive to steroids.* **Sandblasting** mines, “**Eggshell” calcification** of hilar lymph nodes + Macrophages respond to this particle and release broncogenic factors, leading to **fibrosis**... may *_disrupt phagolysosomes and impair macrophages,_* **increasing susceptibility to TB!!!!!!!!!!!!!!!!!!!!** **asymptomatic** (not a pneumoconiosis) **condition found in many urban dwellers exposed to sooty air.** Carbon dust; Massive exposure leads to diffuse fibrosis ('black lung')
name that pneumoconiosis! **“Ivory white,” calci ed, supradiaphragmatic and *_pleural plaques._* pathognomonic of--\> Asbestosis** **aerospace and manufacturing industries.** **Noncaseating granulomas** on histology in the lung, hilar lymph nodes, and systemic organs and therefore *occasionally responsive to steroids. --\>* **Berrylliosis** **Sandblasting.** mines“**Eggshell” calcification** of hilar lymph nodes + Macrophages respond to this particle and release bronchogenic factors, leading to **fibrosis.**.. may disrupt phagolysosomes and impair macrophages, **increasing susceptibility to TB!!!!!!!!!!!!!!!!!!!! --\> Silicosis** **asymptomatic** (not a pneumoconiosis) **condition found in many urban dwellers exposed to sooty air.** **--\> Anthracosis** **Carbon dust;** Massive exposure leads to diffuse fibrosis ('black lung'); --\> **Coal workers’ pneumoconiosis**
165
Mycobacterium scrofulaceum (holy weird as name) causes WHAT
(cervical lymphadenitis in children).
166
Adult male has Dry Tap on BM aspiration and MASSIVE splenomegaly. Tartrate resistnace acid phosphatase +ve what is the diagnosis WHAT ARE THE TWO treatments and their MOA
**Hairy cell leukemia** **CLADRIBINE** (antimetabolite) **PURINE analogue** inhibits DNA pol and causes DNA strand breaks note: the other purine analogue (inhibits DE NOVO purine synthesis is 6MP)
167
what parts of the branchial arch is derived from mesoderm, what part if derived from neural crest?
Branchial arches— derived from **mesoderm (**muscles, arteries) **neural crest** (bones, cartilage).
168
hep b and c are endemic in Sub saharan africa. T or F?
TRUE
169
**bladder exstrophy** is due to what and what **two clincal associations** can you make with it?
Ventral wall defects (tummy side) Developmental defects due to **failure of: *_CAUDAL_* fold closure**—**bladder exstrophy** Exstrophy of the bladder is associated with 1. **Epispadias** 2. **Adenocarcinoma of the bladder** note: Developmental defects due to **failure of:** *ƒ* ***Rostral* fold** closure—**sternal defects** ƒ ***Lateral* fold** closure—**omphalocele & gastroschisis**
170
Up to 25% of PHEOCHROMOCYTOMA cases are associated with germline mutations, name 3 main ones
Up to 25% of cases associated with germline mutations (eg, **NF-1,** **VH**L, **RET** [MEN 2A, 2B]).
171
172
if an HIV pt has a cd count less than 50. he is at risk for getting MAC (mycobacat avium complex). what can you give AS PROPHYLAXIS FOR MAC isoniazid or azithromycin
**dont fall for it!** ISONIAZID **DOES NOT WORK** AGAINST MAC!!!!!! **JUST AZITHRO OR CLARTHRO (MACROLIDES)**
173
For **acute cough,** the most common cause is name 1 For **chronic cough,** the most common causes are name 3
For **acute cough,** the most common cause is • Viral URI For **chronic cough,** the most common causes are * Asthma (most common) * Gastroesophageal reflux disorder (GERD) * Postnasal drip
174
AN **umbilical hernia** (seen at birth) which is reducible adn comes out when crying and also is asymptomatic and resolves spontaneously by around age 3 is classically associated with what disorders and what is the pathogenesis of this disorder.
1. down syndrome 2. hypothroidism (cretinism) 3. Beckwith Wiedman syndrome
175
Copper is a cofactor for what 3 things
Cofactor for 1. **ferroxidase** (binds iron to transferrin), 2. **lysyl oxidase** (cross-linking of collagen and elastic tissue), 3. **tyrosinase** (melanin synthesis)
176
Diapedesis—WBC travels between endothelial cells and exits blood vessel whats on wbc vs endothelium?
PECAM-1 (CD31) **on both**
177
diff btw gen anxiety disorder and adjustment disorder?
GAD last \>6 mos and is NOT brought on by a specifc event, person, place or thing adjustment disorder is \<6 months (\>6 months is the INCITING STRESSOR is chronic) so here we have an actual stessor causing impairment (divorce, illness, etc)
178
WHAT LINES A **PSEUDOCYST**? EPITHELIUM OR GRANULATION TISSUE + FIBROSIS? .......YOU BEST GET THIS RIGHT.
GRANULATION AND FIBROUS TISSUE THATS WHY IS A PSEUDO AND NOT REAL CYST
179
if i say r**homboid crystals** that are **weakly birefringment** under polarized light (**blue when parallel to light)**, what disease process do you think of? what is seen on **xray** in patients with this?
**Calcium pyrophosphate deposition disease** **[PSEUDOGOUT]** X RAY: **chondrocalcinosis** (cartilage calcificaitons) **\*knee** is most affected
180
what the most common COMPLICATION OF **CAUSTIC INGESTION**?
STRICTURE (ESOPHAGEAL)--\> SQUAMOUS CELL CA
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DOC for invasive mucomycosis
amphotericin B
182
what type of drug toxicity is being described: can be due to any cause of decreased GFR, overdose or drug interaction acutely presents with GI sxs Chronically presents with Neuro sxs like ataxia, tremors/fasciculations its MOA is to inhibit the Phophoinositol cascase
Lithium
183
treatment for **ACROMEGALY**
1. **Pituitary adenoma resection.** If not cured, treat with 2. **Octreotide** (somatostatin analog) 3. **Pegvisomant** (growth hormone receptor *antagonist*).
184
**chiari 1 vs 2** KNOW THIS!
due to underdevelopment of Posteror fossa **chiari 1:** more mild (relatively benign) presents at **adolescence,** due to **cerebellar tonsils that extend beyond foramen magnum** into the vertral canal: present like **Paroxsymal occipital HA** (due to meningeal irritation) and **cerebella dys** (ataxia and dizziness) due to compression of cerebella tonsils ---associated with **SYRINGOMYELIA!** **chiari 2**: **more severe**, presents in **neonate** (**tonsils, vermis and medulla even through FM)** -**NON COMM hydrocep** always happens bc of aqueductal stenosis **(compression of medulla**--\> **dysphagia, apnea, and stridor + lumar Associated with: Myelomeningoclee!** --\> lower limb paralysis
185
what are 3 main examples of **Free radical injury** and examples for each category
1. Oxygen toxicity: **retinopathy of prematurity** *(abnormal vascularization)* & **bronchopulmonary** **dysplasia (from Therapeutic supplemental O2 to a baby who has ARDS when its done too quickly!)** ƒ2. Drug/chemical toxicity: **carbon tetrachloride--\>** *causes FATTY change in liver by decreasing protein synthesis (inlcuding apoliopproteins--\>causes fat to be able to come into liver but cant get out)* & **acetaminophen overdose (hepatotoxicity)** ƒ 3. Metal storage diseases: **hemochromatosis (iron)** and **Wilson disease (copper)**
186
List all the CYP450 INHIBITORS **CRACK IM A GQ'SS**
Cimetidine Ritonavir Amiodarone ciprofloxacin Ketoconazole Isoniazid (I**N**H) Macrolides (except azithromycin) Acute Alcohol Abuse Grapefruit juice Quinidine SulfonamideS SSRI's Be careful with **-cyclosporin** (narrow therapeutic index, extenseively met in liver and intestine by CYP3A4)
187
spott necrosis + ballooning degeneration (hepatocyte swelling) + eosinophilic apoptotic bodies in the liver biopsy of a man who recently traveled to s america is the histo you would see in WHAT viral illness? and how would this man classically present one week into the disease?
**HEP A!!!!!!!!!** **NOTE!** the eosinophiic apop bodies are **COUNCILMAN BODOIES!!!!!!!!!** **first is a prodrome: fever**, **ANOREXIA,** N/V**,** malaise THEN CHOLESTATIC signs: jaundice, **DARK COLORED URINE**, LIGHT STOOL, pruitis no carrier state and self limited KNOW THAT HISTO
188
how do bugs confer resistance to MACROLIDES?
well since macrolides work by BINDING TO THE 23S SUBUNIT OF THE 50 S ribosome and blocking translocation if you METHYLATE that 23S rRNA binding site the DRUG CANT BIND AND VOILA!
189
2 causes of Protein-Energy Malnutrition (PEM? PRIMARY and whats the MCC of secondary
1. kwashikor 2. marasmus seconday a**. Secondary PEM** is **common in the elderly** (living alone or in hospitals or nursing homes), chronic alcoholics, the homeless population, and bedridden patients.
190
WHAT IS HOMAN SIGN WHERE IS IT CHARACTERISICALLY SEEN?
**Homan sign**—WHEN DORSIFLEXTIONof foot calf CAUSES PAIN! **DVT!!!!!!!! (inc d-dimer)**
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THESE LABS ARE SEEN WHERE * **INC PTH, INC ALP, INC *cAMP in urine.*** * Most often asymptomatic. * May present with weakness and constipation, abdominal/ flank pain (kidney stones, acute pancreatitis), depression
Primary hyperparathyroidism
192
profound bilateral sensorineural hearing loss in child who experiences syncopal episodes during periods of stress, exercise, or fright. and long QTc, usually greater than 500 msec. diagnosis which disease presents the same but is AD and no deafess?
**Congenital long QT syndrome** **Jervell and Lange-Nielsen syndrome**— autosomal recessive, sensorineural deafness. (AR) **Romano-Ward syndrome**—autosomal dominant, pure cardiac phenotype (no deafness).
193
what effect does restrictive cardiomyopathy have on LV End DIASTOLIC pressure? how does it affect the PV loops?
LV ED**P** increases at the SAME LV ED**V** **moves up and to the left**
194
HOW TO DO DIFF BTW APCKD AND MCKD (multicysitic dysplastic kidney)?
ADCDK follows a clear inheritance pattern. mcdk is congenital and SPONTANEOUS NOT INHERITED adckd have cysts but their renal system is developed just fine mcdk had an issue of the ureteric bud not taking to the metanenpheric mesenchyme so the kidney (ureter, tubules, etc) DO NOT develop propersly AND THIS CAUSES BUILD UP OF URINE AND SUBSEQUENT cysts that dont communicate and fibrosis with lots of cartilage
195
what two opioid analgesic are given for diarrhea as well whats the MOA what drug is given alongside to discourage pts from taking high doses to acheive euphoria or dependence?
loperamide diphenoxylate they bind mu opiod receptors in the GI and SLOW MOTILITY give atropine (causes dry mouth, blurry vision, nausea) so pts wont want to take the opiods in high doses or else they'll get these ae's
196
WBC travels through interstitium to site of injury or infection guided by **chemotactic signals** **what are thees chemotactic signals on the endothelium? (5)** **K-C L I P**
kallikrein LTB4 IL-8 C5a Platelet-activating factor
197
**wickham striate (reticular white lines) in mouth mucosa associated with...**
lichun planus!!
198
what subunits make up fetal vs adult hemoglobin
Fetal Hb (2α and 2γ subunits) Adult is 2a, 2b
199
**In the type of pulmonary HTN caused by Vasoconstriction, is vasoconstriction** is thought to be due in part to **enhanced activity** of name two classic vasoconstrictors and **reduced activity of** name two classic vasodilators.. is there a treatment for this? name 3 if ya can and their MOA!
Vasoconstriction is thought to be due in part to 1. **enhanced activity** of **thromboxane and endothelin-1 (both vasoconstrictors)** and 2. **reduced activity** of **prostacyclin and nitric oxide (both vasodilators).** * **BosENtan:** Competitively **antagonizes ENdothelin-1 receptors--\> DEC.** pulmonary vascular resistance. * **Sildenafil:** I**nhibits cGMP PDE-5** and **prolongs vasodilatory effect of nitric oxide.** * **Epoprostenol, iloprost: PGI2 (prostacyclin) with direct vasodilatory** effects on pulmonary and systemic arterial vascular beds. **Inhibits platelet aggregation.** The increased pulmonary vascular pressure that results from vascular obstruction further injures the endothelium. Injury activates coagulation at the intimal surface, which may worsen the hypertension.
200
what valve is most commonly affected in Rheumatic Heart Disease (Rheumatic Fever)
**Mitral** Mitral\>Aortic\>\>\>\>tricuspid (high-pressure valves affected most).
201
which Pneumoconioses is the **only one** that affects **LOWER lobes**
**Asbestosis**
202
whats the only bug to have protein A as its virulence factor?
staph aureus! prevents opson and phago binds the fc part of IgG
203
WHAT ARE THE BORDERS OF THE FEMORAL TRIANGLE? when the femoral vein runs superior and passes the inguinal ligament, what vein does it officially become?
Inguinal lig (superior) Adductor longus (medially) Sartorius muscle (laterally) EXTERNAL ILIAC VEIN
204
Diagnosis is primarily o**f Guillain-Barré syndrome**. clinical. Similar **acute weakness** can result from myasthenia gravis, botulism, poliomyelitis (mainly outside the US), tick paralysis, West Nile virus infection, and metabolic neuropathies HOW can these usually be distinguished?
* Myasthenia gravis is **intermittent** and **worsened by exertion.** * Botulism may **cause fixed dilated pupils (in 50%)** and **prominent cranial nerve dysfunction** with *_normal sensation._* * Poliomyelitis usually **occurs in epidemics.** * Tick paralysis causes **ascending paralysis** but *_spares sensation._* * West Nile virus causes headache, fever, and **asymmetric** flaccid paralysis but *_spares sensation._* * Metabolic neuropathies occur with a **chronic metabolic disorder.**
205
whats the substrate that inhibits CPT-1 in carnitine shuttel during FA degrad and what is the use of this type of reg?
**Malonyl CoA** inhibits **CPT-I,** thus preventing the entry of long-chain acyl groups into the mitochondrial matrix. Therefore, when **fatty acid synthesis is occurring in the cytosol** (as indicated by the presence of malonyl CoA), the newly made palmitate cannot be transferred into the mitochondria and degraded. so that FA synthesis and degrad are not happening at the same time?
206
does Sirolimus (Rapamycin) work by 1. Preventing **IL-2 transcription (production)** or 2. Preventing **response** to IL-2. whats its main MOA in contrast to cyclosporine and tacrolimus how does siroliums differ? what its main AE?
**its an: mTOR inhibitor;** **binds FKBP.** Blocks T-cell activation and B-cell differentiation **by preventing response to IL-2.** **calcineurin: Calcineurin inhibitor; binds *_cyclophilin. --_*Blocks T-cell activation by preventing IL-2 *transcription.*** **tacrolimus: Calcineurin inhibitor; binds *_FK506 binding protein (FKBP)._*** **Blocks T-cell activation by preventing IL-2 *transcription.*** ***both tacrolimus and calcineurin are NEPHROtox but SIROLIMUS is NOT NEPHROTOXIC! its causes pancytopenia, insulin resistance & hyperlipidemia;*** ***\*\*\*\*\*\*******Kidney “sir-vives.”* Synergistic with** **cyclosporine.**
207
which one of the following require a SEGMENTED GENOME (as they relate to Viruses). Give an example fo a virus that would take place in this. recombination reassortment phenotypic mixing complementation
REA**SS**ORTMENT requires a VIRUS WITH A **S**EGMENTED GENOME S for S eg: **influenza**
208
a LOW maternal AFP is characterisically seen in
DOWN SYNDROME
209
linelozind, whats a bad AE
serotoninc syndrome!!!
210
**Adenomas (Conn)** caused by an **adenoma**, usually **unilateral**, of the glomerulosa cells of the adrenal cortex or, more rarely, by adrenal hyperplasia. In **adrenal hyperplasia,** which is more common among older men, **both** adrenals are overactive, and no adenoma is present.
Hypernatremia, hypervolemia, and a hypokalemic alkalosis may occur, causing episodic weakness, paresthesias, transient paralysis, and tetany. Diastolic hypertension and hypokalemic nephropathy with polyuria and polydipsia are common note: Low levels of both **PRA (Plasma renin activity (**) and **aldosterone** suggest **nonaldosterone mineralocorticoid excess** (eg, due to licorice ingestion, Cushing's syndrome, or Liddle syndrome).
211
**HVA and VMA** (catecholamine metabolites) in ***_urine!_*** ***_Bombesin and NSE ⊕_****_._* Associated with **overexpression of *_N-myc oncogene_****_._* Classidfied as an **APUD tumor** (amine precursor uptake decarboxylase (APUD) associated with a characteristic *_Pareneoplastic syndrome_* that is seen also seen in adults (when adults have Smal cell CA) **diagnosis?**
**Neuroblastoma** * Most common tumor of the adrenal medulla in children, **usually \< 4 years old.** * Originates from **neural crest cells.** Occurs anywhere along the sympathetic chain Can also present with **opsoclonus-myoclonus syndrome (“dancing eyes-dancing feet”).** **NOTE: you can say the "adult" version of this is tumor is Pheochromocytoma which is MORE likely to present with HTN than this neuroblastoma**
212
how heat stable are ENDOtoxins
stable at 100 deg for 1 hour
213
match the histo description with the type of Hep virus (choose from HEP A, B or C 1. Granular **eosinophilic “ground glass”** appearance; [cytotoxic T cells mediate damage] 2. **Lymphoid aggregates** with focal areas of **macrovesicular steatosis** 3. Hepatocyte swelling, **monocyte** infiltration, **Councilman bodies (refer to attached image for description of a councilman body)**
1. Granular **eosinophilic “ground glass”** appearance; [cytotoxic T cells mediate damage] = **HEP B** 2. **Lymphoid aggregates** with focal areas of **macrovesicular steatosis = HEP C** 3. Hepatocyte swelling, **monocyte** infiltration, **Councilman bodies = HEP A**
214
which of the following cytokines are critical for the **formation and maintenance of granulom**as (eg: in TB). Pick alll that apply a. IL-4 b. IL-2 c. IL-12 d. IFN gamma e. IL-6 f. TNF-a qID: 301
c. IL-12 (induces t helper cells differentialtion into the Th1 subtype) d. IFN gamma (produced by **mature Th1 cells** which then activate macs, improving their abiity to kill ingested mycobacteria. f. TNF-a (produced by **activated macs** to recruit additonal monocytes and macs to the area) macrophage mediated immunity to Mycobacterium Tb is led by Th1 helper cells (you go down this pathway **when IFN-gamma \> IL-4)**
215
which is the **childhood tumor** that is Derived from **remnants of Rathke pouch?** where in the brain is it located? **supra or infratentorial**? **how does it present** **whats commonly seen of it on imaging**
-Most common childhood **supratentorial tumor.** - Derived from **remnants of Rathke pouch.** * *-Calcification** is common on imaging -May be confused with pituitary adenoma (both can cause **bitemporal hemianopia)**
216
increased wall stress on **right-**-\> HEMATOCHEZIA=associated with what GI pathology? increased wall stress on **left**--\> HEMATOCHEZIA=associated with what GI pathology?
increased wall stress on **right-**-\> HEMATOCHEZIA=associated with **ANGIODYSPLASIA (Most often found in cecum, terminal ileum, ascending colon)** increased wall stress on **left**--\> PAINLESS HEMATOCHEZIA=**DIVERTICULOSIS** [Many false diverticula of the colon, commonly sigmoid. Caused by intraluminal pressure and focal weakness in colonic wall. Associated with low- FIber diets.
217
POMC is enzymatically cleaved to produce what 3 things?
b-endorphins (delta receptor) ACTH MSH
218
[HERITABLE] Pulmonary arterial hypertension is caused by:
an inactivating mutation in **BMPR2 gene** (normally inhibits vascular smooth muscle proliferation); poor prognosis. note: Other causes include drugs (eg, amphetamines, cocaine), connective tissue disease, HIV infection, portal hypertension, congenital heart disease, schistosomiasis.
219
why is **OSMOTIC DIARRHEA** + A **DECREASED STOOL pH** seen in **LACTOSE INTOLERANCE**?
OSMOTIC DIARRHEA bc lactose attracts water into the small bowel A **DECREASED STOOL pH** bc **BACTERIA** in the gut **FERMENT** THE LACTOSE INTO LACTIC ACID! NOTE: **Lactose hydrogen breath test: ⊕** for lactose malabsorption **if postlactose breath hydrogen value rises \> 20 ppm** compared with baseline.
220
Aortic narrowing near insertion of ductus arteriosus (**“juxtaductal”**). Associated with **bicuspid aortic valve**, other heart defects, and **Turner syndrome.** **Hypertension in upper extremities and weak, *delayed pulse in lower extremities (brachial-femoral delay*).** **name that congenital heart defect**
Coarctation of the aorta
221
whats the difference btw WET and DRY beri beri (for fun, deficiency in what vitamin causes this)?
**Dry beriberi**—polyneuritis, **symmetrical** muscle wasting. **Wet beriberi—**high-output cardiac failure (dilated cardiomyopathy), edema.
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what type of behavior is HTLV associatd with?
(associated with I**V drug abuse)** Adults present with **cutaneous lesions;** especially affects populations in J**apan, West Africa, and the Caribbean.** **Lytic bone lesions, hypercalcemia**.
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i got this wrong note the broad based budders (which i didnt initially see and note **NEUTROPENIA)** thing **fungal**
**answer is AMPHOTERICIN** general funal treatment: Treatment: Fluuconazole or itraconazole for **local infection;** ***_amphotericin B for systemic infection._*** ***_H-B-C-P_*** **are the SYSTEMIC MYCOSIS** **side note:** Systemic mycoses can form granulomas (like TB) but cannot be transmitted person-to-person (unlike TB).
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the upper part of the vagina developes from the \_\_\_\_\_\_\_\_\_ while the lower part develops from the \_\_\_\_\_\_\_\_\_
1. Paramesonephric (MULLERIAN) duct 2. urogenital sinus
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what is the GHRH analog used to treat HIV associated lipodystrophy?
**tesamorelin!**
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cyst brain lesion + immuno**COMPETENT** patient + native to **Central/South america** + seizures/ intracranial HTN you should be suspicious of what and treat how?
**neurocysticercosis** treat with **albendazole**
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**"periventricular calcifications"** are seen in a baby with unique
a **CONGENITAL CMV i**nfection! (transmitted to mom via **sexual contact or TRANSPLANT)** Hearing loss, seizures, petechial rash, “blueberry muffin” rash, **periventricular calci cations** *not periventricular plaques assoc with MS*
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what is the most common predisposing condition for native valive IE (infective endocarditis) IN DEVELOPED NATIONS? how about in DEVELOPING nations?
**MITRAL VALVE PROLAPSE WITH REGURGITATION** (deposits on valves can become colonized by microorganism (eg: strep viridans) during episodes or bacteremia (eg: following a dental extraction). NOTE: IE--\> embolic stoke (middle cerebral artery)--- (common complication) **RHEUMATIC HEART DISEASE (under developed nations)**
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72-year-old woman comes to the physician because of a 6-month history of increased bruising on her forearms. She appears alert and well nourished. Physical examination shows extensive wrinkling, **scaly erythematous patches** on the face, and **irregularly shaped brown macules** on the face and forearms. There are ecchymoses in various stages of healing on both forearms; the ecchymoses are more numerous on the right side. Laboratory studies, including a complete blood count and coagulation studies, are within the reference ranges. She has noticed no bleeding from her gums after brushing her teeth. Which of the following is the most likely cause of the ecchymoses in this patient? A. Exocytosis of lymphocytes B. Extensive solar elastosis C. Impaired platelet function D. UV destruction by Langerhans in epidermis
B. Extensive **solar elastosis** Histo feature seen in **Actinic keratosis!** Premalignant lesions caused by sun exposure. Small, rough, erythematous or brownish papules or plaques . Risk of squamous cell carcinoma is proportional to degree of epithelial dysplasia.
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**Richter transformation is**—(*when the type of cancer whose name you are trying to guess*) undergoes transformation into an *aggressive lymphoma,* most commonly **diffuse large B-cell lymphoma (DLBCL**).
Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL)
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what factors cause a shift of the Oxygen-hemoglobin dissociation curve of the curve to the **right?** **does a right shift mean that unloading o2 is favored or not?**
"Right shift—When curve shifts to the right, DECREASED affinity of Hb for O2 (**facilitates unloading** of O2 to tissue). ACE BATs right handed" **A**cid **C**O2 **E**xercise 2,3-**B**PG **A**ltitude **T**emperature
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82-year-old woman comes to the physician because of constant severe lower abdominal pain and fever for 24 hours. Laproscopic examination shows severe diverticulosis and perforated diverticulitis. In spite of appropriate therapy she dies 2 days later. Liver autopsy shown. Which of the following is the primary component of the material shown on the hepatic surface?
**FIBRIN!!!!!**
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what **vitamin deficiency** is being described Hemolytic anemia **acanthocytosis** muscle weakness posterior column and spinocerebellar tract demyelination.
**Vitamin E DEFICIENCY** DONT BE FOOLED. THIS IS SIMILAR TO VIA B12 DEFICIENCY BUT IS DIFFERENT BC HERE WE ARE LACKING: megaloblastic anemia hypersegmented neutrophils INC serum methylmalonic acid levels.
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DRUGS THAT CAUSE: **[DILE]** Drug induced lupus Erythematosus (SLE-like syndrome) **"SHIPP-E"**
Sulfa drugs, **\*\*Hydralazine,** **\*\*Isoniazid,** **\*\*Procainamide (class 1A antiarrythmic)** (note: Procainamide is metabolize by hepatic ACETYLATION: therefore, SLOW acetylators are at higher risk of DILE **Phenytoin,** **Etanercept** **SHIPP-E**
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what are the lab presentations in primary (psychogenic) polydipsia Psychogenic polydipsia is found in patients with **mental illnesses, most commonly schizophrenia** The most common presenting symptom is tonic-clonic seizure, found in 80% of patients.
**HYPOnatremia** bc they be drinking so much water (UNLIKE central or nephro DI where they have HYPERnatremia!!!) INITIAL low urine OSMOlarity (but then quickly responds to water depriv and urine inc in osmolarity). also, urine will not change in osm after ADH is given bc max endogenous ADH has already been used The most common presenting symptom is tonic-clonic seizure, found in 80% of patients.[21] Psychogenic polydipsia should be considered a life-threatening condition, since it has been known to cause severe hyponatraemia, leading to cardiac arrest, coma and cerebral oedema. Euvolemic hyponatremia: In euvolemic (dilutional) hyponatremia, total body Na and thus ECF volume are normal or near-normal; however, TBW is increased. Primary polydipsia can cause hyponatremia only when water intake overwhelms the kidneys' ability to excrete water. Because normal kidneys can excrete up to 25 L urine/day, hyponatremia due solely to polydipsia results only from the ingestion of large amounts of water or from defects in renal capacity to excrete free water. Patients affected include those with psychosis or more modest degrees of polydipsia plus renal insufficiency. Patients with acute psychogenic water drinking are able to concentrate their urine during water deprivation. However, because chronic water intake diminishes medullary tonicity in the kidney, patients with longstanding polydipsia are not able to concentrate their urine to maximal levels during water deprivation, a response similar to that of patients with partial CDI. However, unlike CDI, patients with psychogenic polydipsia show no response to exogenous ADH after water deprivation. This response resembles NDI, except that basal ADH levels are low compared with the elevated levels present in NDI. After prolonged restriction of fluid intake to ≤ 2 L/day, normal concentrating ability returns within several weeks.
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EUVOLEMIC (normal volume, normal BP) HYPONATREMIA is classically seen in....
SIADH!!!! Characterized by: * ƒ Excessive free water retention * ƒ Euvolemic hyponatremia with continued urinary Na+ excretion which is protective to keep BP and vLs in check via the following mechanisms: \*\*\*Body responds to water retention with aldosterone and ANP and BNP---\> INC urinary Na+ **secretion--\>** normalization of extracellular uid volume---\> euvolemic hyponatremia.
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what disease is this **HONEYCOMB LUNG** **CYCLICAL LUNG INJURY:** repeated cyles of lung injury and wound healing (diffuse fibrosis) increased collagen deposition **-digital clubbing**
**IDIOPATHIC PULMONARY FIBROSIS (IPF)** aka: Cryptogenic fibrosing alveolitis a type of restrictive lung disease affecting the interstitium
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what two ethnicitis are at inc risk for pancreatic adenomcarcinoma?
jews african americas
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VACTERL association specifically refers to the abnormalities in structures derived from what embryonic entity? what does vacterl stand for?
**MESODERM!** * *V**ertebral defects * *A**nal atresia * *C**ardiac defects **T**racheo-Esophageal stula **R**enal defects **L**imb defects (bone and muscle)
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**RECURRENT** C DIFF INFECTIONS whats the DOC whats its MOA? is it safe in pts allergic to penicillin?
**FIDAXOMICIN!!!!!!!!!!!!** its i**nhibits the sigma subunit of RNA POL**!!!!! its a macrocylic antibiotic **SAFE!!!!**
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what are the **two complications** of **Vit EXCESS?**
1. in pts at high risk for iron overload, VIT C can cause IRON OVERLOAD (bc its job is to convert fe 3+ to fe 2+ (which can then be absorped in intestine) 2. can cause ca2+ oxalate stones bc (vit C--\> metabolized into Oxalate!
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in this disease, **Aplastic anemia,** which is **Caused by failure or destruction of myeloid stem cells, is due to:** **(DNA repair defect** causing bone marrow failure); also **short stature**, incidence of tumors/leukemia, c**afé-au-lait spots**, **thumb/radial defects.** **what disease is this? that is causing this aplastic anemia?** **whats the other main disease caused by defective DNA repair** cannot repair dna via Nonhomologous end joining which rings together 2 ends of DNA fragments to repair double-stranded breaks. No requirement for homology. Some DNA may be lost
Nonhemolytic, normocytic anemia **Fanconi anemia** **ataxia telangiectasia is the other one!**
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during Tight-binding—whats on wbc vs endothelium? is there a deficiency associated here?
ICAM-1 (CD54)- on **endothelium** CD11/18 integrins (LFA-1, Mac-1) on **WBC** (defective in **leukocyte adhesion deficiency type 1** (CD18 integrin subunit)
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**CD5+ve** associated with Coombs +ve hemolytic indolent
Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) **SMUDGE CELLS!!!!!!**
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* **“brown tumor”** consisting of **osteoclasts and deposited hemosiderin from hemorrhages**; causes **bone pain**) where is this seen?
**DUE TO PRIMARY HYPERPARATHYROIDISM** **Osteitis brosa cystica**—cystic bone spaces lled with brown brous tissue (“brown tumor” consisting of osteoclasts and deposited hemosiderin from hemorrhages; causes bone pain).
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the most common **acquired** inflammatory neuropathy. **autoimmune attack** of ***_peripheral myelin ("PRIMARY AFFERENTS")_*** due to **molecular mimicry,** inoculations, and stress, but no definitive link to pathogens. **Flaccid weakness predominates** in most patients; it is always more prominent than sensory abnormalities WHATS THE **DIAGNOSIS**? WHAT WOULD BE TYPICALLLLY SEEN ON **LABS**? _HERES THE NBME Q ON THIS_ 24-year-old man with **3-day progressive numbness** of **both feet ascended** to thighs. Last 24 hours, numbness and tingling of hands. PE **ataxic gait**. **Deep tendon reflexes diminished in upper extremities and absent in knees and ankles. Vibration and joint position absent in fingertips and feet bilaterally**. Mild weakness distal upper extremities ad moderate weakness of lower extremities. Structure involved?=**MYELINATED PRIMARY AFFERENTS**
**Guillain-Barré syndrome**. [an Acute in ammatory demyelinating polyradiculopathy) Associated with infections (eg, **Campylobacter jejuni,** viral)---\> autoimmune attack of peripheral myelin Findings: CSF protein with **normal cell count** (**albuminocytologic dissociation**). Complication of the labs findings: protein may cause **papilledema.**
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* **5-HT3 antagonist**; DECREASES vagal stimulation. **Powerful *central-acting antiemetic.*** * ***whats this drug? and what KIND of nausea does it treat?*** * ***main SERIOUS AE?*** **VERSUS:** * ***D2 receptor antagonist. resting tone, contractility, LES tone, motility.*** * *WHATS THE NAME OF THIS DRUG* * *WHAT ARE ITS USES?* * *WHEN IS IT CONTRAINDICATED?*
**Ondansetron!!!** * Control vomiting ***postoperatively and in patients undergoing cancer chemotherapy.*** * **AE: Long QT interval WHICH predisposes to torsades de pointes.** **Metoclopramide!!!** * **Diabetic and postsurgery gastroparesis,** * **Antiemetic** * **AEL Parkinsonian effects, tardive dyskinesia. Restlessness, drowsiness, fatigue, depression, diarrhea.** * **Drug interaction with digoxin and diabetic agents.** * **Contraindicated in patients with small bowel obstruction or Parkinson disease (due to D2-receptor blockade).**
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whats the pathogenesis behind the levels of CK as they relate to the "MYOPATHY" seen in Hyper and Hypothyroidism? when are CK levels inc and when are they normal and why?
* **Hypo**thyroid myopathy (proximal muscle weakness, **INCREASED CK)** * \*this is bc **DEC** THYROID HORMONE I**NC THE PERMEABILITY OF SKELETAL MUSCLE MEMBRANES** SO CK LEAKS INTO BLOOD AND IS MEASURED AS INC IN SERUM. VS. * **Thyrotoxic (hyperthyroidism)** myopathy (proximal muscle weakness, normal CK) * you get the myopathy bc of INC PROTEIN CATABOLISM (but no inc in CK bc the membranes of the skeletal muscle are ok).
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Results in inappropriate laughter (“happy puppet”), seizures, ataxia, and severe intellectual disability. Diagnosis? Paternal or maternal imprinting? gene from dad or mom is normally silent? Paternal or Maternal gene is deleted/mutated?
**AngelMan syndrome** **Paternal** Imprinting gene from **dad** is normally silent and **Maternal** gene is deleted/mutated.
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FINDINGS in this diseaes: - HF - S3 - systolic regurgitant murmur dilated heart on echocardiogram, balloon appearance of heart on CXR. what am i describing? what are the 9 main etiologies? **ABCCCD-PSH**
Dilated cardiomyopathy **A**lcohol abuse, **w**et Beriberi, **C**oxsackie B viral myocarditis, **c**hronic **C**ocaine use, **C**hagas disease, **D**oxorubicin toxicity, **p**eripartum cardiomyopathy. **s**arcoidosis, **h**emochromatosis, **\*Systolic dysfunction ensues. \*Eccentric hypertrophy**
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old lady comes in bc of muscle weakness and tenderness on her shins. she has **LOW ca2+** and **HIGH PTH** **what does she have?** **osteoporosis** **osteomalacia** **metastatic breast cancer** **primary hyperparathyroidsm**
NOT OSTEOPOROSIS, labs are NORMAL in osteoporosis **OSTEOMALACIA!!!!!!!!!**
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which hematologic cancer shows REAL REAL LOW LAP and commonly accelerates and transforms to "blast crisis" and can be treated with imatinib
Chronic myelogenous leukemia!!!!!!! NOTE: **Very low LAP** as a result of low activity in malignant neutrophils (vs benign neutrophilia [leukemoid reaction], in which LAP is INC ). Responds to **bcr-abl tyrosine kinase inhibitors** (eg, **imatinib**).
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why would loss of Ca2+ in the ECM lead to loss of cell-cell adhesion?
bc Cadherins which bind to cadherins on neighboring epithelial cells are CALCIUM dependent.
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which TCA is DOC for OCD
clomipramine
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diff btw dry and wet beri beri
seen in malnourished areas dri: muscle and neuro + NO EDEMA wet: tachy and high output cardiac failure (dilated CM) + WITH EDEMA
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whats the #1 RF for transitional cell CA? but then what are the other RFs?
SMOKING!!!! #1 **Pee SAC:** Phenacetin, Smoking, Aniline dyes Cyclophosphamide.
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"germ tubes" what are they and what bug are they associated with and at what temperature?
Germ tubes: **short outgrowth, non-septate germinating TRUE hyphae**. They are ½ the width and 3 – 4 times the length of the cell from which they arise. When cells of **Candida** are incubated in serum at **37C** for **2-4 hours** Candida albicans produce short, slender, tube like structures called **germ tubes** Note: A germ tube test is a diagnostic test in which a sample of fungal spores are suspended in animal serum and examined by microscopy for the detection of any germ tubes.[2] It is particularly indicated for colonies of white or cream color on fungal culture, where a positive germ tube test is strongly indicative of Candida albicans.[2]
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whats the most abundant aa in collagen? lysince proline glycine
GLYCINE!! dont you dare say proline
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what two cancers are pts with ataxia telangiectasia at risk for? whats the pathogenesis of this disease? what is CHARACTERISTICALLY ELEVATED IN THE SERUM AND WHAT IS CHARACTERISTICALLY DECREASED?
primary cns lymphoma leucemia ATM gene is mutated and leads to INACTIVATION OF tp53--\> which leads to failure to repeair DNA double stranded breaks---\>CELL CYCLE ARREST INCREASED AFP! DECREASED IgA, G & E! you see lyphopenia and cerebellar atrophy!
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cutaneous erythmatous marks in a fern-leaf pattern are known as\_\_\_\_\_\_and are an indication of\_\_\_\_\_\_\_\_
**LICHTENBURG FIGURES** LIGHTNING INJURY (can have 2nd degree burns too) (ESPECIALLY IF THE PT IS FOUND ON WET GROUND OR UNDER A TREE AND UNCONSCIOUS )
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whats the hallmark cell in a wilms tumor?
**blastema!!!!!!!!!!** = (immature kidney mesenchyme), primitive glomeruli and tubules, and stromal cells
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DOC for **OCD** (3) (note: if there is a specific drug in one of the 3 classes for OCD, name it)
SSRIs venlafaxine (SNRI) **clomipramine (**TCA)\*\*\*\*\*\*\* know this association!
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which immunosuppresant drug is a RF for Invasive CMV?
Mycophenolate mofetil moa: Reversibly inhibits **IMP dehydrogenase,** preventing purine synthesis of B and T cells.
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if you fuck up your LGN (lateral geniculate nucleus) WILL YOUR PUPIILARY LIGHT REFLEX be affected? like will you not constrict?
NOOOOOO!!!!!!! LGN is involved in your VISION pathway with CN II NOT CNIII in the PNS pathway for pupillary constriciton if you messed up the LGN you woudlnt be able to too on one side of the eye on both eyes, like Left LGN injury--\>vision in BOTH EYES on one side being affected/lost)
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cholera and what e coli exotoxin have the same MOA
cholera and ETEC (one of its enterotoxins is the Heath Labile (LT) that also inc camp---\> diahrrhea
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SOMEONE HAS A HEMISECTION AT T8 (BROWN SEQUARD), WHAT LEVELS WILL HAVE **LOSS OF PAIN (PINPRICK!) AND TEMP?**
CONTRALAT LOSS OF PAIN AND TEMP 2 LEVELS BELOW AND EVERYTHING UNDER! **EG: INJURY AT T8** **LOST OF PAIN AND TEMP** ONE OR TWO SEGMENTS **BELOW!!!**! LIKE AT **T9/T10!!**
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what vitamin does the following: Antioxidant (protects RBCs and membranes from free radical damage).
Vitamin E (tocopherol/tocotrienol)
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the following disease course: in a **young and otherwise healthy pt** severe respiratory distress (fatigue, **progressive dyspnea)**, atypical chest pain --\> cyanosis and **RVH**/and or dilation of the RV (after long standing disease)--\> **death from decompensated cor pulmonale.** what disease process should be suspected
**Pulmonary hypertension** **[if the pulmonary HTN is due to Primary processes affecting the lung] its called "PAH (Pulmonary arterial hypertension )**
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whats the MOA for Iron poisoning and how it causes damage?
**Cell death due to peroxidation of membrane lipids.**
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aspergillus Causes **invasive aspergillosis,** especially in immunocompromised and those with chronic granulomatous disease what are specific RF's to getting this? what does aspergillus look like that makes it unique (aside from its ACUTE 45 angle branching)...
Major risk factors include • **Neutropenia!!!!!!!!!!!!!!!!** • Long-term high-dose corticosteroid therapy • Organ transplantation (especially bone marrow transplantation) • Hereditary disorders of neutrophil function (eg, chronic granulomatous disease) • AIDS Aspergillus sp tends to infect open spaces, such as pulmonary cavities caused by previous lung disorders (eg, bronchiectasis, tumor, TB), the sinuses, or ear canals (otomycosis). UNIQUE: Produces **conidia in radiating chains** at end of conidiophore E .
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there is a patient with subacute endocarditis in a previously damaged heart valve who gets subacute endocarditis, what is the unique feature about this bug that allows for adherance to human surfaces and subsequent infection? whats the bug and some of its basic properties?
dextrans (insoluble extracell polysaccarhide), which bind to brin-platelet aggregates on damaged heart valves and tooth enamel. **STREP VIRIDANS** {(*Streptococcus mutans* and *S mitis*) and subacute bacterial endocarditis at damaged heart valves (*S sanguinis*) ) Gram ⊕, α-hemolytic cocci. Resistant to optochin, [differentiating them from S pneumoniae, which is α-hemolytic but is optochin sensitive]
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what renal disease can Wilsons diseae lead to?
Fanconi Syndrome due to deposition of Cu in the PCT
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YOU BETTER NOT FREAKIN GET CONFUSED BETWEEN **ASPIRIN** AND **ACETMINOPHEN** ANYMORE!!!!!!!! HOW WOULD LAB VALUES IN both be DIFFERENT?
**ASPIRIN = INC BLEEEEEDING TIME!!!** **ACETMINOPHEN = INC PT!!!!!!!**
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WHICH EMBRYONIC WEEKS ARE extremely susceptible to teratogens
EMBRYONIC PERIOD weeks 3-8 **Neural tube formed by neuroectoderm** and closes by week 4. **Organogenesis.**
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* unresponsiveness of kidney to PTH hypocalcemia *despite PTH levels.* * Characterized by **shortened 4th/5th digit**s, * **Short stature.** * **Autosomal dominant.** * Due to **defective Gs protein α-subunit** **causing end-organ resistance to PTH**. what is this disease? is it inherited from mom or dad? describe the disease the disease thats like this but thats inherited from the OTHER parent.
* **Pseudohypoparathyroidism type 1A** **(Albright hereditary osteodystrophy)** * Defect must be inherited from **mother due to imprinting. ​** * **Pseudo*pseudo*hypoparathyroidism—**physical exam features of **Albright hereditary osteodystrophy but _*without end-organ PTH resistance*._** * **Occurs when defective Gs protein α-subunit is inherited from *_father_*.**
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what nerve roots and nerver innervate the doral interosse which abdunt and adduct fingers
**abduction and adduction of fingers (interossei),** **Ulnar (C8-T1) !!!!**
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shifting sinusoidal waveforms on ECG is how it presents and is caused by: drugs, DEC K+ & DEC Mg2+
Torsades de pointes
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WHAT ARE THE LEVELS OF **EPO** like in **Polycythemia vera (JAK2 mutation)?**
out of all the type of poycythemia, its is the only one that has **DECREASED** **EPO due to negative feedback suppressing renal EPO production**
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which eye condition has painless porgressive vison loss beginning with NIGHT blindness bc its affects rods first?
retinitis pigmentosa seen spicules arond macula
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diaphragm innervates which two parts of the parietal pleura
diagphragmatic and mediastinal parts
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what GI disorder is AKA: "pancreatic CHOLERA" what else does this disease cause
**VIPoma!** its also called WDHA ("whaddha" like "water" stands for Watery Diarrhea HypoKalemia and Achlorhydria bc it causes secretory diarrhea (3L/day) not osmotic like lactose bc that would repsond to diet mod and not inflamm diarr bc that has pus or blood its SECRETORY (do to inc relase of na, cl and water into bowel)
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TTP. talk about it. whats the deficiency, does it have Coombs'-negative hemolytic anemia. or Coombs'-positive hemolytic anemia?
involves nvolve **nonimmunologic** platelet destruction Coombs'-**negative** hemolytic anemia!!!!!!!! Inhibition or deficiency of **ADAMTS 13** (**vWF metalloprotease)---\>** degradation of vWF multimers. **Pathogenesis: INC** large vWF multimers--\> platelet adhesion--\> **platelet aggregation and thrombosis.** **Labs:** 1. **Schistocytes** 2. **LDH** **Symptoms**: ***_pentad_*** of: 1. neurologic 2. renal symptoms 3. fever 4. thrombocytopenia, 5. microangiopathic hemolytic anemia. **Treatment:** plasmapheresis, steroids.
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Triad of polyarthralgias tenosynovitis (eg, hand) dermatitis (eg, pustules). seen in ? what is the other presentation of this condition? it has 2
**Gonococcal arthritis** **—STI that presents as either** **1. purulent arthritis (eg, knee)** **2. triad of polyarthralgias, tenosynovitis (eg, hand), dermatitis (eg, pustules).**
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what genetic principle is displayed by **alpha 1 antitrypsin**? hint Blood groups A, B, AB also share this.
**co dominance** when both alleles contribute to the phenotype of the heterozygote
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which antimicrobial should be avoided by bc displaces BR from albumin and can lead to kernictuerus?
SUlfonamides
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what two arteries mainly come off the external iliac artery? which one courses **more medially**?
the **superficial epigastric artery** and the **inferior epigastric artery** (more medial)
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which primary immune disorder is characteriized by **Low to normal IgG, IgM.** **INCREASED IgE, IgA.** **Fewer and smaller platelets.**
**Wiskott-Aldrich syndrome** Mutation in **WAS gene**; T cells **unable to reorganize actin cytoskeleton**. X-linked recessive. **WATER**: **W**iskott-**A**ldrich: **T**hrombocytopenia, **E**czema, **R**ecurrent infections. **risk of autoimmune disease and malignancy.**
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what are the levels of **CHOLESTEROL in hypo and hyper thyroidism**?
**Hypo**thyroidism---\> **Hyper**cholesterolemia (due to **DEC** LDL receptor expression) **Hyper**thyroidism--\> **Hypo**cholesterolemia (due to **INC** LDL receptor expression)
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how can you tell diff btw lipoma and lipsarcoma?
liposarcomas have a SCALLOPED BORDER to the nuclear membrane (they are pleiomorphic)
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high fever, black vomitus, and jaundice. (eosinophilic apoptotic globules) on liver biopsy (these actually have a name) what infection is this?
**Yellow fever virus** A **flavivirus** (also an arbovirus) transmitted by **Aedes mosquitoe**s A . Virus has a monkey or human reservoir. Symptoms: **high fever, black vomitus, and jaundice.** May see ***_Councilman bodies_*** (eosinophilic apoptotic globules) on liver biopsy. affects **ZONE 2** of liver **HAS Live attenuated vaccines**
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nephritic syndrom with HYPOCOMPLEMENTENEMIA PERSISTENTLY LEVELS OF C3 ARE DECREASED! IT CAN ONLY BE ONE THING
TYPE II MPGN (CALLED DENSE DEPOSIT DISEAE) NOTE: T**YPE 1** WITH **PAS +VE STAIN AND H&E** shows ***_TRAM TRACK ON LM_*** appearance due to mesangial ingrowth **splitting the GBM** (dont confuse with the **"spike and dome** appearance seen in MN where you realy thick GMB thickening so thick due to NEW GBM proliferation)
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if a patient has **high AST: ALT \>2** and also pancreatitis whats (another) lab value you can use that is specific to the LIVER (to rule out, pancreatitis caused by something else like gallstones which the the #1 cause, alcohol is #2) blood sugar MCV WBC count
MCV!!!! bc in alcoholism [EVEN WITHOUT A FOLATE DEF] YOU CAN STILL SEE macrocytosis!!!! **MCV\>100!!!!!!**
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the **C**ommon **C**arotid artery and proximal part of internal **C**arotid artery are derivatives of **aortic arch?**
**3rd!** C is the 3rd letter in the alphabet!
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purines and pyrimidine bases that are to be used for DNA syntheses are denovo synthesized where RER nucleus cytoplasm golgi SER
CYTOSOL!!!! not the nucleus! and this deNOVO synthesis requires CO2 so if a probe following CO2 was used you would see it in the CYTOSOL
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in a vignette, if there ever is a **baby (1-3 days old)** who presents with **billious vomiting** and an **ABSENT JEJENUM/ILEUM** (a type of intestinal atresia)...what is the **first CAUSE** you should think of? whats the **embryological derivative?**
the cause is **VASCULAR INJURY!!!!!** **MIDGUT structures are listed so SUPERIOR MESENTERIC VESSELS ARE DISRUPTED--\> segmental resorption/atresia/bowl discontinuity-** **not** failure to recanalize (that would be DUODENAL ATRESIA)==\> double bubble sign on xray and it presents in 1-2 days of life
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Dihydropyridine CCB's affect capillary hydrostatic Pressure in what way and why?
Inc capillary hydrostatic Pressure by impairing normal arteriolar dilation--\> non inflammatory edema (excess accum of transudative fluid in teh interstitial tissues)
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what are the MAIN AE of CHLOROQUINE/HYDROXYCHLOROQUINE which are used in MALARIA (but also RA! just not the best at the RA)
RETINOPATHY AND skin pigmentation pruiris
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whats the relationship btw MAC (mean alveolar concentratin and), potency and lipid solubility of a drug (anesthetics) how does onset of action & recovery/elimination play a role?
**increased lipid solubility** = **MORE POTENT**= **small MAC** (potency is **INVERSLY** related to MAC) why? bc MAC is the **MINIMAL ALVEOLAR CONCETRATION** (of INHALED anesthetic) required to precent 50% of subjects from moving in response to noxious stimuli (eg: skin incision) the **more lipid soluble (and blood soluble)-**-\> slow inc in PP (arterial tension)/LARGE amount needed to SATURATE blood--\> slower equilibrium with brain---\> S**LOWER ONSET OF ACTION & RECOVERY/ELIMINATION**
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whats the pathogenesis of the lab values seen in Wilsons disease? namely High urinary Copper Low serum Ceruloplasmin
Wilsons is a genetic defect impairs copper transport. The **impaired transport decreases copper secretion into the bile**--\> copper overload and resultant **accumulation in the liver.** Then copper diffuses out of the liver into the blood, then into other tissues; which begins at birth. The impaired transport also interferes with incorporation of copper into the copper protein ceruloplasmin, thus decreasing serum levels of ceruloplasmin **BC CERULOPLASMIN IS STABALIZED BY COPPER SO WITH OUT COPPPER ITS HALF LIFE IS DECREASED SO THE LEVEL OF IT ARE DECREASED IN THE SERUM** Hepatic fibrosis develops, ultimately causing cirrhosis. It is **most destructive to the brain (eg, dysarthria, dystonia, tremor, parkinsonism)**,but also damages the **kidneys and reproductive organs** and causes **hemolytic anemia.** Some copper is deposited in Descemet's membrane of the cornea, causing **Kayser-Fleischer rings.**
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* **what bug is this?** Gram ⊝, flagellated, comma shaped, oxidase ⊕, grows *in alkaline media.* Endemic to developing countries. Produces its characteristic illness via **enterotoxin that *permanently activates Gs, cAMP.*** * **is it acid labile** (dies in acid) or **acid-stable**? * **does it require a large or small requires large inoculum?**
**Vibrio cholerae** Sensitive to stomach acid (acid labile); requires **large** inoculum (high ID50) unless host has gastric DECREASED acidity (then itll be ok bc it likes all the alkaline things)
305
Lithium is almost identical to what ion (Na+, K+ phosphate or Ca2+) and is excreted almost entirely by the\_\_\_\_\_\_\_\_
Na+ KIDNEY
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**WHICH VITAMEN** CAN BE USED AS TX FOR for **methemoglobinemia** (in addition to methylene blue) by **reducing Fe3+ to Fe2+.**
**vitamin C**.
307
what levels of the following promote gall stones cholesterol phosphatidyl choline bilirubin bile acids
HIGH chol HIGH BR LOW BILA ACIDS AND LOW PC = GALL STONE FORMATION BC AS BILE GETS SECRETED INTO THE GALL BLADDER THE JOB ON THE BILE SALTS AND PC TO MAKES IT SOLUBLE FOR EXCRETION WHEN THERE IS MORE BILE DUE TO INC CHOLES, THE LOAD FOR THESE BILE ACIDS AND PC IS TOO MUCH AND BILE FORMS ROCKS
308
contents of the **femoral triangle from** LATERAL--\> MEDIAL? is it beneath or above the inguinal ligament?
Lateral to medial: **Nerve-Artery-Vein**- Lymphatics. REMEMBER: **You go from lateral to medial to find your NAVeL.** **BELOW the inguinal ligament**
309
why is posterior urethral valves a malformation only seen in males?
bc its from a **MALFORMATION OF THE WOLFFIAN (MESONEPHRIC DUCTS!)**
310
when is an IVC filter indicated in a pt?
when pt has DVT or pulmonary embolism who what contraindications to anticoagulation
311
* Adults present with cutaneous lesions; * especially affects populations in J**apan, West Africa, and the Caribbean.** * **OSTEOLYTIC** bone lesions, hypercalcemia. * associated with IV drug use diagnosis? whats the prognosis?
BAD PROGNOSIS (death within months-a year) Adult T-cell lymphoma Caused by **HTLV** (associated with IV drug abuse)
312
whats the difference btw: **Repression vs suppression** are they immature or mature defense mechanisms?
**Repression**=IMMATURE **Involuntarily** withholding an idea or feeling from conscious awareness. -blacking out the 10 years in your life you were raped vs **Supression**=MATURE **Intentionally** withholding an idea or feeling from conscious awareness; *temporary.* * -*choosing to not worry about clinical rotations until its a more appropriate time to think about it bc other things are more important - putting Bobby and boys in general on the way back burner bc school is more important
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Caused by DECREASED hypocretin (orexin) production in lateral hypothalamus. what does hypocretin (orexin) do?
**Narcolepsy** **hypocretin orexin maintains wakefullness**
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PT HAS RA is prescribed the first line drug for it develops mucosal oral ulcers pulm fib inc ast and alt what drug
**methotrexate!** **its doesnt just cause BM suppression!** its PREFERENTIALL INHIBITS RAPIDLY GROWING CELLS LIKE INFLAMMATORY AND NEOPLASTIC CELLS (WHICH LEAD TO CHARACTERISTIC BM SUPPRESSION--WHICH leucovorin reverses) **BUT ALSO....** **CELLS WITH RAPID TURN OVER LIKE** **GI (ORAL/GI ULCERS), LIVER (HEPATOX: CIRRHOSIS AND FIBROSIS)**
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chronic cholestatic syndromes characterized by **patchy inflammation, fibrosis, and strictures** of the ***_intrahepatic and extrahepatic bile ducts._*** Progression obliterates the bile ducts and leads to cirrhosis, liver failure, and sometimes cholangiocarcinoma & gall bladder cancer. which Biliary tract disease is this?
Primary sclerosing cholangitis
317
mets from RCC is most common to what two places? LN or hematogenous spread?
RCC spreads hematogenously (exceptino to carcinome spreads via LN rule) t**o LUNG #1** bone also adrenals and liver
318
Decreased or **loss of pubic hair**+failure to lactate+cold intolerance in a woman should make you think of what
these are all things that are controlled by stuff made by the pituitary so SHEEHAN! Sheehan syndrome—**ischemic infarct of pituitary following postpartum bleeding;** * pregnancy-induced pituitary growth susceptibility to hypoperfusion (bc not enough blood supply to keep up with the growth:( meu * Usually presents with failure to, lactate, absent menstruation, cold intolerance
319
* **Nodular GOITER seen in...what 4 main cases** * **Smooth/difuse GOITER seen in...what 4 main cases**
**Nodular** Toxic multinodular goiter Thyroid adenoma Thyroid cancer Thyroid cyst **Smooth/difuse GOITER seen in...** Graves disease Hashimoto thyroiditis Iodine deficiency TSH-secreting pituitary adenoma
320
whats the logic behind **"INDUCING"** someone to have methemoglobinemia during **CYANIDE poisoning**? what do you use to induce it? which complex does CN inhibit in the ETC making it a mitochondrial toxin?
**Induced methemoglobinemia** ## Footnote you use: **nitrites,** **followed by thiosulfate--\> which oxidizes Fe2+ to Fe3+= and there you have it: Methemoglobinemia**) ratioale; Oxidized form of Hb **(ferric, Fe3+)** that does **not** bind O2 as readily, but h**as affnity for cyanide. So it can scavenget it bascially by preferentially binding it over O2.** note: **CN** is a mitochondrial toxin that specifically inhibits **complex IV** in the ETC.
321
List all the things that taking Thiazide diuretics increase in the body (looking for 5 things) what 3 things do they Lower
increase serum: CA2+ uric acid=Hyperuricemia (gout) glucose cholesterol TAGs Lower Na+, K+ and Mg2+ metabolic alkalosis note-Thiazide use is implicated in lithium toxicity in bipolar patients.
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what the connection btw ALCOHOL and GOUT
**Acute attack** tends to occur after a large meal or **alcohol consumption** (alcohol metabolites **compete for same excretion sites in kidney as uric acid--**\> **DECREASED uric acid** secretion and **subsequent buildup in blood).**
323
what is the hallmark of REVERSIBLE cell injury
Cellular swelling (secondary to changes in ion concentration and the influx of water)
324
name 5 presenting features that should make you think multiple myeloma and why
325
FEBRILE SEIZURES VS HEAT STROKE IN A KID WHATS THE DIFF AND HOW DO YOU HANDLE THEM DIFF?
LOOK HERE TOO FOR MORE EXPLANATION
326
content of what **Amino acid** best reflects collagen synthesis? what what step of collagen synthesis requires Vit C (where does it occur?)
**GLYCINE** (bc **collagen is 1/3 Glycine)** remember the **alpha chains** are: **GLY-X-Y** **X-Y (are Proline or Lysine)** **STEP 2** or the **HYDROXYLATION STEP needs VIT C** (this is a **post-transciptional step** where **Proline and Lysine are hydroxylated** **in the RER**!!!!)
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**diagnosis?**
**Charcot-Marie-Tooth disease** Also known as hereditary motor and sensory neuropathy (HMSN). Group of progressive hereditary nerve disorders related to the defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath. Typically **autosomal dominant** inheritance pattern * associated with **foot deformities (pes cavus),claw hands, steppage gait, skinny legs, loss of sensation in feet,** l**ower extremity weakness and sensory deficits.**
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Synovial uid infammatory (WBC \> 2000/mm3). Involves **MCP** **PIP** **wrist** ***not DIP or 1st CMC.*** **"Ulnar devations (swan neck deformity) and "Boutonniere"**
RA
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Chromogranin A ⊕, neuron-specific enolase ⊕. \ what cancer is this? May produce: **ACTH** (Cushing syndrome) **SIADH** Antibodies against presynaptic Ca2+ channels **(Lambert- Eaton myasthenic syndrome)** Antibodies against: neurons (paraneoplastic myelitis, encephalitis, subacute cerebellar degeneration). **Amplification of myc oncogenes common**. Managed with chemotherapy +/– radiation.
**Small cell (oat cell) carcinoma**
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polymoid mass of "clear grapes" coming out of the vagina of a 4 year old (or someone less than 4) immunocytochem +ve for?
Vaginal tumors **Sarcoma botryoides (embryonal rhabdomyosarcoma variant)** **spindle-shaped cells; desmin ⊕.**
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do **most** meiotic non disjunction cases resulting in Down syndrome happen in MEIOSIS 1 or MEIOSIS II mainly?
**meiosis 1 !!!!!!!** the RFLP will how **3 bands!** one from dad t**wo from mom**---\> this indicates that the baby inherited BOTH of her homologous chromosomes so separation of them did not occur in meiosis one like normal note if the baby had 2 band: 1 from dad of NORMAL thickness and one from mom but it is THICKER then this indicates non disjunction in MEIOSIS 2!!! baby inherits both sister chromatids, which will produce equal size restriction fragments but TWICE THE NORMAL AMOUT FOR ARE FATTER BANDS LOOK IN NOTEBOOK!
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**Anti-mitochondrial antibody ⊕**, IgM. Autoimmune reaction lymphocytic infiltrate + granulomas--\> destruction of **intralobular bile ducts (only!).** **is this histo similar to any particular type of histo seen in transplant pts?**
**Primary biliary cirrhosis** **histo same as GVHD histo**
333
this disease Initially **presents like serum sickness** (which means what sxs exactly?) what does it look like on HISTO? Which types of immune cells mediate the damage? what is the mode of transmissin? does it have potential to progress to carcinoma?
**HEPATITIS B VIRUS** I Initially like **serum sickness** **"PLAN PR FFHV"** **P**roteinuria **L**AD **Arthralgia** **N**eutropenia (not -philia) **Pruitic Rash** **Fever** **F**ibrinoid **N**ecrosis **H**yptocomplementenemia **V**asculitis (vasculitis) **HISTO**: Granular eosinophilic “**ground glass”** appearance **cytotoxic T cells mediate damage** **Mode of transmission:** Parenteral (**Blood)**, sexual (**Baby- making**), perinatal (**Birthing**) **YES**, may progress to carcinoma
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whats the cause of Pulmnoary HTN in cases of obstructive sleep apnea or living in high altitude?
**hypoxemic vasoconstriction**
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Paradoxical embolism (which originate in the systemic venous circulation (lower or upper extremeties and then enter the arterial circ via an intracardiac or intrapulmonary shunt) is commonly associated with what 3 congenital heart defects
**Patent foramen ovale (btw RA & LA)** **ASDs** (atrial L--\>R shunts can faciliate paradox emb due to periods of **transient shunt reversal (!!!!!)** **to a R--\>L** during eg: **coughing or straining**)--\> RA--\> LA--\> LV-\> aorta--\> paradoxical embolism (can go to MCA and bam, stroke) VSDs (also: Large pulmonary arteriovenous malformations)
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**Rosenthal fibers—eosinophilic, corkscrew fibers** what childhood brain tumor is this
**Pilocytic (low-grade) astrocytoma** **Most often found in posterior fossa (eg, cerebellum). May be supratentorial.** **GFAP ⊕. Benign** **good prognosis.**
337
list all X linked recessive disorders! **O**blivious **F**emale **W**ill **O**ften **G**ive **H**er **B**oys **H**er x-**L**inked **D**isorders
Ornithine transcarbamylase deficiency, Fabry disease, Wiskott-Aldrich syndrome, Ocular albinism, G6PD deficiency, Hunter syndrome, Bruton agammaglobulinemia, Hemophilia A and B Lesch-Nyhan syndrome, Duchenne (and Becker) muscular dystrophy. Note-Female carriers can be variably affected depending on the percentage inactivation of the X chromosome carrying the mutant vs normal gene.
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an opportunistic infection caused by inhaling spores of the mold (fungus in question here) the spores **invade blood vessels,** causing hemorrhagic necrosis and infarction. Symptoms may be those of **asthma, pneumonia, sinusitis, or rapidly progressing systemic illness.**
**INVASIVE Aspergillosis** Treatment is with **voriconazole, amphotericin B (or its lipid formulations), caspofungin, itraconazole, or flucytosine.** Fungus balls may require surgical resection. Recurrence is common.
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Migratory (superfiicial) Thrombophelbitis, known as **Trousseau Syndrome** [*A PARANEOPLASTIC SYNDROME OF HYPERCOAGULABILIT*Y] is seen in pts with what **3 types of cancer** for the most part
**#1 Pancreatic adenocarcinoma** [also **Lung and colon** adenocarcinoma]
340
what diseae process SHOULD be suspected in patients with **significant exertional dyspnea** who are **otherwise relatively healthy** and have **no history or signs of other diseases known to cause pulmonary symptoms.**
**Pulmonary hypertension !!!!!!**
341
look at the peekchur **Bamboo spine (vertebral fusion)** **More common in males.** **seen in...** **use the mnemonic _U-S-A_ to name the 3 main manifestations**
**Ankylosing spondylitis** **U**veitis, **Symmetric** involvement of **spine and sacroiliac joints----\> ankylosis** *_(joint fusion)_*, **A**ortic regurgitation.
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what part of the PDH complex does arsenic inhibit?
Lipoic acid!
343
when you lose consciouness, can you hear someone talking to you? important when trying to distinguish btw types of seizures!
NO!!!!!!!!! so you are not resonsive **gradual** loss of consciousness is **NOT seizure or a cardiac even**t but instead is **metabolic like Hypoglycemia** **a sudden loss of consciousness is**
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what does Francisella tularensis need to grow? whats special about the way it stains
**NEEDS CYSTEINE!!!!**
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Rash on palms and soles
Coxsackie A, 2° syphilis, Rocky Mountain spotted fever
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WHICH GRAM +VE enterotoxin is not destroyed rapidly, as is most common, at 60 degrees?
staph enterotoxin is not killed
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**Dimorphic,** **cigar-shaped budding yeast that grows in branching hyphae** with **rosettes of conidia;** causes local pustule or ulcer initially (after trauma to skin makes it enter) then has nodules **along draining lymphatics (ascending lymphangitis)**. Disseminated disease possible in immunocompromised host. diag and treatment
**Sporotrichosis!!!** Sporothrix schenckii **rose gardener’s” disease** Treatment: **itraconazole** or **potassium iodide.**
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what are the two principal site of HEME synthesis?
1. IMMATURE rbc (erythrocyte precursors)/ERYTHROBLASTS 2. hepatocytes note: after dividing a bunch. IMMATURE rbcs LOST not only their nucleus but their MITONCHONDRIA as well so they CANNOT MAKE HEME ANYMORE (need mitochondria for FIRST AND LAST 3 STEPS OF HEME SYNTHESIS!) SO MATURE RBCS live to 120 days all the while unable to make heme
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28-year-old woman comes to the physician because of a 4-day history of palpitations, severe neck pain, fatigue, and malaise. Her pulse is 120/min and regular. Physical examination shows a diffusely tender, mildly enlarged thyroid gland. There is no exophthalmos. Serum studies show a thyroid-stimulating hormone concentration of 0.01 μU/mL. Which of the following is the most likely diagnosis? A. Factitious thyrotoxicosis B. Graves disease C. Subacute granulomatous thyroiditis D. Thyroid abscess E. Toxic multinodular goiter
**Subacute granulomatous thyroiditis (de Quervain)** **severe neck pain!!!!!!!!!!!!!!!!!** you missed this before bc you thought HYPERTHYROID bc of sxs. BUT this can be HYPERthryroid AT FIRST and then is HYPO. BUT THE PAINNNNN!!!!!!!!!!! main point. dont overlook shit like that.
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during Margination and rolling, whats on the WBC (neutrophil) and whats on the endothelium?
E-selectin P-selectin ON WBC Sialyl-LewisX ON ENDOTHELIUM
351
During placement of a central venous catheter, which two out of these three sites preferred due to less risk of infection? internal jugular vein subclavian vein femoral vein
**internal jugular vein** **subclavian vein** femoral vein {NOT preferred bc has higher risk of infection)
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what are the two inherited **CONGENITAL LONG QT** disorders of myocardial repolarization, typically due to ion channel defects; risk of sudden cardiac death (SCD) due to torsades de pointes. Includes:
whats they KEY diff? one has deafness, Jervel and Lange, the other doesnt, Romano Ward -one is AD, the other is AR.
353
which type of chiari malformation (1 or 2) is **Spina Bifida Cystcica (myelomeningocele)**?
**CHIARI MALFORMATION 2**! presents in childhood bc more severe. not 1. (1 is milder and more common); presents at adolescence or later
354
list all the **neuroendocrine tumors:**
Group of neoplasms originating from **Kulchitsky and enterochromafin-like cells**. Occur in various organs eg, **thyroid:** medullary carcinoma; **lungs**: small cell carcinoma; **pancreas**: islet cell tumor; **adrenals:** pheochromocytoma). Cells contain **amine precursor** uptake decarboxylase **(APUD**) and secrete different hormones (eg, 5-HIAA, neuron-specific enolase **[NSE]**, **chromogranin A**).
355
whats the treatment for **Disseminated Lyme Disease**
Doxyclycline Ceftriaxone
356
how do levels of ghrelin differ in prader willi vs after gastric bypass
well seeing as ghrelin is made my the stomach, gastric bypass **DECreases ghrelin** and in prader they are fat so **ghrelin inc**
357
**Anti-GpIIb/IIIa antibodies** **what disease?** **​whats MOA of this diseae?** **what are labs like?**
**Immune thrombocytopenia** ## Footnote **Anti-GpIIb/IIIa antibodies-**-\> splenic macrophage consumption of platelet-antibody complex. -Commonly due to *viral illness.* ***Labs:** **megakaryocytes INC** on bone marrow biopsy.* ***Treatment:** steroids, IVIG, splenectomy (for refractory ITP).*
358
what MOA allows Desmopressin to be used in the treatment of vWb deficiency and (mild) Hemophilia A
Desmopressin releases vWf stored in the endothelium & increases circulating factor VIII \*vWf enhances clotting in 2 way: 1. augments platelet binding (which helps form the initial plt plug). 2. stabalizes factor VIII (vwf is non covalently attached to VIII)
359
Smooth parts (outflow tract) of left and right ventricles are derived from what embryonic structure?
Bulbus cordis
360
WHY does Fetal Hb have a higher affinity for O2 than adult Hb, driving diffusion of oxygen across the placenta from mother to fetus?
**DECREASED** affinity of HbF for **2,3-BPG**----\> **INCREASED** affinity of Hb for O2!
361
inguinal bubo two bugs do this
**Lymphogranuloma venereum C trachomatis (L1–L3)** Infection of lymphatics; **painless** genital ulcers, **painful** l**ymphadenopathy (ie, buboes)** **and YERSINA PESITS (one sided inguinal bubo after hiking) + HA and fever ---\> leads to plague**
362
what is ALWAYS the relationship between Ferritin and TIBC?
**ALWAAYYYSSS opposite!!!!!** ferritin is stored iron and tibc indirectly measures transferrin which is what transports Fe3+ state iron in the blood; so if there is iron stores, transferrin wouldnt need to be going arond in the blood looking for more iron to transport (bc either its locked away or bc there just isnt enough iron to transport even.
363
is alcohol a RF for panc adenocarcinoma? is smoking? are the ducts or acini affected in this cancer?
alcohol is NOT a direct RF!!!!!!!!!!!!!!!! but smoking iSSSsSSSSSSSSsssss DUCTSSSSS!!!!!!!!!!!
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**Down syndrome** is most commonly a consequence of maternal **meiotic nondisjunction.** what type of congenital defects are most associated with this disease (give specific embryological mechanism)
AV septal defect (**endocardial cushion defect**), VSD, ASD
365
whats the difference btw: are they immature or mature defense mechanisms? **Reaction formation** **vs** **Sublimation**
**Reaction formation:** when you have an obsession with something thats probably unacceptable **so you _(unconsciously choose to)_ do the extreme opposite** ***-sex addict becomes priest*** **vs** **Sublimation:** Consciously, replacing an *unacceptable* wish with a course of action that is similar to the wish but **does not conflict with one’s value system** **-angry teen dances off her stress and excels**
366
Same MOA as amphotericin B. **Topical** **use only** as **too toxic for systemic use**. “Swish and swallow” for oral candidiasis (thrush); topical for diaper rash or vaginal candidiasis.
Nystatin
367
the 6th AORTIC arch gives rise to which 2 strucutres
Proximal part of pulmonary arteries and (on left only) **ductus arteriosus.**
368
**Halothane (an inhaled anaesthetic) can cause what changes most commonly in what organ.** **how would a patient present?** **would PT or PTT be prolonged?**
**Focal to massive hepatic necrosis (LIVER!)** Pts has **"inhaled anesthetic hepatoxicity"** **PT** is prolonged bc failure of liver to be able to synthesize factor VII (which has the shortest half life of all the coag factors)
369
IN A PT WITH isolated Mitral stenosis, how is the LV end diastolic pressure affected? what are all the conseq of MS?
its usually UNCHANGED!!!! or even a little decreased bc of the stenotic valve, but the pressures before it are high so if a gives you an altered LVEDV there is likely aortic valve involvement! (AR)
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Is "squatting" helpful or does is exascerbate Tet of Fallot? and why?
Squatting helps: INCREASES Peripheral SVR (afterload), DEC right-to-left shunt, improves cyanosis.
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Affects the elderly; idiopathic; CSF pressure elevated **only episodically;** does not result in increased subarachnoid space volume. **Expansion of ventricles** **Characteristic magnetic gait (feet appear stuck to oor). ​** **1. urinary incontinence** **2. ataxia** **3. cognitive dysfunction (sometimes reversible).**
“Wet, wobbly, and wacky.” ## Footnote **Normal pressure hydrocephalus**
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Acute generalized cortical infarction of **both** kidneys. Likely due to a combination of vasospasm and DIC is called **Diffuse cortical necrosis and is highly associated with**
Associated with obstetric catastrophes (eg, **abruptio placentae**), **septic shock.**
373
what are the **2** main **complications** of **Neonatal respiratory distress syndrome**? what are RF's?
1. **PDA** due to Persistently low O2 tension 2. **Necrotizing enterocolitis:** Seen in premature, formula-fed infants with immature immune system. Necrosis of intestinal mucosa (primarily colonic) with possible perforation, which can lead to p*neumatosis intestinalis, free air in abdomen, portal venous gas.* **RFs:** prematurity, maternal diabetes (due to fetal inc insulin), C-section delivery ( dec release of fetal glucocorticoids).
374
in cirhosis when you have thrombocytopenia and intrinsic plt dysfunction causing plts to be, for exampls, \<50,000. how will the BT and PT change?
TRICK! PT will NOT CHANGE!!! but BT increases. dont fall for this. in a pt with plt sequestration due to hypersplenism and subsequent thrombocytopenia who has cirrhosis and also due to less thrombopoeitin being made in liver....you are only affected Plts NOT coag cascade directly so labs for PT and PTT wil be normal. this is how you would diff it from a factor 7 def due to cirrhosis where the PT would be increased. (note: in this case, they will tell you, that after giving Vit K, the PT had NO change, thats bc the liver is so sick it cant make enought factors 2,7,9,10 to even attempt to correct coag
375
why is vit b12 deficiency called subacute COMBINED
due to ascending (DCML) AND descending (Corticospinal) look at attahced
376
as it relates to the oncogenesis from HPV strains, viral protein _____ degrades p53, the tumor suppressor protein regulating the cell cycle) viral protein\_\_\_\_inhibits the retinoblastoma gene product Rb
viral protein \_**E6**\_\_\_\_ degrades p53, the tumor suppressor protein regulating the cell cycle) viral protein\_\_**E7**\_\_inhibits the retinoblastoma gene product Rb
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what disease has the following histology? eosinophilic casts resembling thyroid tissue (thyroidization of kidney).
Chronic pyelonephritis
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Nitrites (eg, from dietary intake or polluted/high altitude H2O) and benzocaine cause poisoning by oxidizing Fe2+ to Fe3+.
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what are 3 main complications of ## Footnote Coal workers’ pneumoconiosis silicosis asbestosis
risk of **cor pulmonale** **cancer** **Caplan syndrome** (**rheumatoid arthritis** and **pneumoconioses** with **intrapulmonary nodules**).
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flank pain radiating to the groin with a ballotable flank mass that develops one week POST a pelvic surgery like removing pelvic nodes or hysterectomy points to what charateristically
hydronephrosisi due to uteric obstruction during surgery
381
WHAT THE MOST COMMON CAUSE OF CORONARY SINUS DILATATION?
PULM HTN!
382
**seizures!** **partial (aka: FOCAL)** ones. how do you categorize them and what differentiates them from **generalized (Diffuse)** one?
**partial/focal ones** **[can be simple PARTIAL or complex PARTIAL!!!!]** Affect **single area** of the brain (like **one not both hemispheres!**). Most commonly originate in **medial temporal lobe**. Often **preceded by seizure aura**; can secondarily generalize. Types: **ƒ Simple** partial (**consciousness intact**)— motor, sensory, autonomic, psychic..PATIENT **CAN STILL HEAR YOU** AND **IS RESPONSIVE** BUT **A SINGLE PART OF THEIR BODY** LIKE THEIR ARM OR FACE (ARM AND FACE IF TEMORAL ETC)...BUT **NOT THEIR WHOLE BODIES!!!!!!!** ƒ **Complex** partial (impaired consciousness) PATIENT **CANNOT** HEAR YOU AND IS **NOT** RESPONSIVE BUT A **SINGLE PART OF THEIR BODY** LIKE THEIR ARM OR FACE (ARM AND FACE IF TEMORAL ETC)...BUT **NOT THEIR WHOLE BODIES!!!!!!!** **generalized affect your whole body like both hemispheres**
383
this red safranin O stain is staining red an area that has a large content of.......
TYPE 2 COLLAGEN= cartilage (including HYALINE), vitreous body and nucleous pulposus safranin O stains CARTILAGE!!! photo is of articular cartilage = HYALINE GLASS LIKE COLLAGEN
384
adrenal **medulla** is derived from **cortex** is derived from
**chromaffin cells are here!** (arise from neural crest). cortex from **MESOderm!!!!**
385
is insomnia a knows AE of Benzos? How about Muscle rigidity?
NERP. dont fall for it. they are used as a hypnotic so HELP insomniacs they are even used as a muscle RELAXANT so no to the muscle rigidity
386
hemochromatosis leads to what kind of cardiomyopathy
dilated but also restrictive (mostly dilated)
387
whats the main difference with how someone gets cysticercosis or neurocysticercosis vs just intestinal tapeworm?
cysticercosis or neurocysticercosis is from EATING POOP FROM HUMAN FECES CONTAMINATED WITH EGGS (FROM A CARRIER WHO HAD INTESTINAL TAPEWORM)----NOOOTTTTT FROM UNDERCOOKED PORK! intestinal tapeworm is from EATING LARVAE CYTS IN UNDERCOOKED PORK!
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**“onion skin**” bile duct fibrosis--\> alternating strictures and dilation with **“beading**” of **intra- and extrahepatic bile ducts on ERCP** **Associated with ulcerative colitis. p-ANCA ⊕. IgM. Can lead to *_2° biliary cirrhosis_*. risk of cholangiocarcinoma and gallbladder cancer.** **Classically in middle-aged men with IBD.**
Primary sclerosing cholangitis
389
what 3 drugs are mainly associated with the production of SIADH as an AE? **CCS** what are the names of the **neurons** in which ADH is synthesized with in the Supraoptic Nuclei of the hypothalamus?
**CCS** **CARBAMAZEPINE** **CYCLOPHOSPHAMIDE** **SSRIs** MAGNOCELLULAR NEURONS
390
which congenital heart defects are know to have a risk of leading to paradoxical emboli (venous thromboemboli that enter systemic arterial circulation)
atrial septal defects such as: 1. Patent foramen ovale 2. ASD (atrial septal defect)
391
main diff btw janeway lesions and osler nodes IN INFECTIVE ENDO? 3 BIG diff!
**OSLER NODE** ARE **IMMUNOLOGICAL** (IMMUNE COMPLEX DEPOSITIONS); **JANE WAY** ARE JUST **VASCULAR** DUE TO SEPTIC EMBOLI FROM THE VEGETATIONS AT HEART VALVE **OSLERS HURT**!!!! **JANEWAY IS NICE AND THEY DONT HURT!** **JANEWAY** ARE **AT PALMS AND SOLES** **OSLERS** ARE AT THE P**AD OF FINGERS ON FEET AND HANDS**
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note
Reverse transcriptase is a **very inaccurate DNA polymerase;** it makes lots of mistakes. **High mutation rate** causes HIV to constantly change, a serious problem for making a vaccine.
393
**Autosomal dominant** disorder most common in **Asian males.** ECG pattern of **pseudo-right bundle branch block** and ***_ST elevations in V1-V3._*** INC risk of ventricular tachyarrhythmias and SCD. Prevent SCD with implantable cardioverter-de brillator (ICD). **DIAGNOSIS?**
**Brugada syndrome** It is caused by a defect in **the sodium channel** of the cardiac cell membrane. **Syncope and cardiac arrest: Most common clinical manifestations; in many cases, cardiac arrest occurs during sleep or rest** **Nightmares or thrashing at night** **Asymptomatic, but routine ECG shows ST-segment elevation in leads V1-V3** **Associated atrial fibrillation (20%) [1]** **Fever: Often reported to trigger or exacerbate clinical manifestations**
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* pts thinks she is super fat and is consumed by this thought even though she is actually thin * she binges 3x a week for greater than 3 months * then she feels like shit about herself * then she fasts * and also exercises 3 hours a day * her BMI is 23 which does she have? **body dysmorphic disorder** **bulimia nervosa** **binge eating disorder**
believe it or not **BULIMIA NERVOSA** why? bc to meet the criteria you need to binge eat at least once per week for at least 3 months and have SOME TYPE OF COMPENSATORY BEHAVIOR (EG: LAXATIVE, PURGE (VOMIT), EXERCISE A TON, FAST) **YOU DONT HAVE TO VOMITTTTTTT** - note: in binge eating disorder there is NO compensation - and in body dysmorphinc you can be meeting criteria for an eating disorder like this and simply be given a BDD diag. duh.
395
'LYMPHOCYTES WITH CYTOPLASMIC PROJECTIONS" what the frick does this **actuallly** mean.
the cells characteristically seen in **HAIRY CELL LEUKEMIA** mature b cell tumor also can be described as filamentous hair like projections" **either way, PROJECTIONS FROM THE CYTOPLASM!!!!!!!!!!!!!!!!**
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6-year-old girl with 15-minute history of severe shortness of breath. Diagnosed with throat tumor 3 years ago. RR 32. PE nasal flaring. Laryngoscopy shows multiple raised, finger-shaped lesions from vocal cords and epiglottis. Lesions excised and shows finger-shaped fibrovascular cores lined with benign squamous epithelium. **Causal virus and diag?** **a) CMV b) EBV C) HSV1 d)HSV2) e) HPV type 6** **note: disease is most commonly found in children**
**_laryngeal papillomatosis_** * **Laryngeal papillomatosis, also known as recurrent respiratory papillomatosis or glottal papillomatosis or associated with condyloma acuminata, is a rare medical condition** * **caused by a human papillomavirus (HPV) infection of the throat.** * **Laryngeal papillomatosis is caused by HPV types 6 and 11, in which benign tumors form on the larynx or other areas of the respiratory tract. These tumors can recur frequently, may require repetitive surgery, and may interfere with breathing.** * **Laryngeal papillomatosis causes assorted tumors or papillomas to develop over a period of time.** * **Without treatment it is potentially fatal as uncontrolled growths could obstruct the airway.**
397
FAILURE OF THE PROCESSUS VAGINALIS TO OBLITERATE CAN CAUSE WHAT TWO CONGENTICAL DEFECTS IN MALE REPRO
1. HYDROCELE (smal hole so just fluid leaks) 2. INDIRECT INGUINAL HERNIA (larger hole so abd contents can leak it) [BOTH have SAME PATHOGENISIS, just different extremes of "obliteration"]
398
399
where do beta lactamases accuulate while exiting (only gram -ve bacteria) obvi this location or spot is absent in gram +ve bacteria
b lactasmases are hydrolytic enzymes that accumulate in the **PERIPLASMIC SPACE =** space btw cytplasmic membrane and **outer membrane** in gram NEGATIVE bacteria
400
what is unique about **syncytiotrophoblasts** in the placenta that **decrease chance of attack by maternal serum?**
**they LACK MHC-1** expression! so smart. **normally** MHC-1 is Expressed **on all nucleated cells** and hence, not expressed on RBCs
401
35-year-old woman with infertile, receive injection of contrast material into cervix. On hysterosalpingogram (shown), contrast material (indicated by arrows) also seen in peritoneal cavity, which explain this finding?
**- Spillage of contrast which *_normal_***
402
what are the **4 cases** hcg is **inc** and what are the b in which hcg is **dec**
**hCG is INC** in multiple gestations hydatidiform moles choriocarcinomas **Down syndrome** **note:** Large cell lung carcinoma *secretes bhcg!* **hCG is DEC** in ectopic/failing pregnancy, Edward syndrome, and Patau syndrome.
403
which B cell, cell surface marker (protein) is the receptor for EBV? CD19 CD 20 or CD 21
CD21
404
**all antiDEPRESSANTS can induce WHAT** in pts taking them (can take up to a few weeks)
**freakin MANIA (like you would see in bipolar 1)** pts comes in wanting to spend recklessly, grandiosoty, racing thoughs, dec need for sleep. pressure speech....MANIC sxs! remember DIG FAST mnemonic
405
if a person has hemibalismus whats a common cause of it? what type of vascular injury could have occured and what would be the 2 main RFs for this?
a person who gets **hemibalismus (wild LARGE AMPLITUDE, flinging movements on one side)** is often due to a LACUNAR STROKE which itself is due to 1. LONG STANDING (BENIGN) HTN 2. DIABETES MELLITUS! \*BC BOTH LEAD TO **HYALINE ARTERIOSCLEROSIS**---\> small LACUNAR **infarct** **so LACUNAR infarct secondary to hyalnie arteriosclerosis (which dev due to long standing benign HTN and DM)**
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DIAGNOSE THIS!!!! The condition is often first noticed in infants with fat **malabsorption**, steatorrhea, and failure to thrive. Intellectual disability may result. Because vitamin E is distributed to peripheral tissues via VLDL and LDL, most affected people eventually **develop severe vitamin E deficiency.** Symptoms and signs include visual changes from **slow retinal degeneration (bilateral) retinitis pigmentosa** , sensory neuropathy, **posterior column signs,** and cerebellar signs of **dysmetria, ataxia,** and spasticity, which can eventually lead to death. ***_NOTE!!!!!!!!!!_*** ***_RBC acanthocytosis is a distinguishing feature on blood smear._***
**Abetalipoproteinemia** (Bassen-Kornzweig syndrome): This **autosomal recessive** condition is caused by mutations in the gene for microsomal triglyceride (TG) transfer protein, a protein **critical to chylomicron (B48) and very-low-density lipoprotein (VLDL) formation (B100).** Diagnosis is made by the **absence of apoprotein B (apo B) in plasma!!! so the two above apolipoproteins are missing!**
407
how the hell is the **lac operon regulated**? how does **HIGH lactose** and **LOW glucose** affect it?
**High lactose--\>** unbinding of the repressor protein from repressor/operator site (also tells **allolactose** to bind the repressor so it doesnt try to pull any sneaky moves)--\>**transcription.** **Low glucose--\>** adenylate cyclase (adenylyl cyclase) activity generation of cAMP from ATP--\> activation of catabolite activator protein (CAP)-\> **transcription.** ƒ
408
what is the DOC for abolishing (and diagnosing) a PSVT (paroxysmal SVT)
**adenosine** inc K out of cells, hyperpolarizes the cell and dec amt of Ca2+ inside adenosine itself is blocked by theophylline and caffeine (both are adenosine receptor antag)
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WHAT TYPE OF CELL JUNCTION PLAY AN IMPORTANT ROLE IN LABOR CONTRACTIONS? (in parentheses are the proteins that make up the individual cell junctions themselves) TIGHT JUNCTIONS (CLAUDINS. OCCULUDINS) ADHERIN JUNCTIONS (CLAUDINS) GAP JUNCTIONS (CONNEXINS)
**GAP JUNCTIONS (CONNEXINS)**
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immuno**compeTENT** patients gets **ORAL candidiasis** eg: kid who is asthmatic can get it from taking oral or inhaled steroids, neonates bc underdev immune system. **whats the DOC?**
**NYSTATIN!!!!!** swish n swallow
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Adrenal cortex (derived from ) and medulla (derived from ).
adrenal cortex from mesoderm chromaffin cells of adrenal medulla derived from neural crest
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which parasite that loves the biliary tract can give you PIGMENTED GALLSTONES AND CHOLANGIOCARCINOMA
clonorchis sinesis tx: prazi its a trematode fluke
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yo. know that **interferion alpha and beta** are DIFFERENT and have a different MOA than I**nterferon GAMMA**
414
what are the two main substances associated with causing ## Footnote **Methemoglobinemia**
**Nitrites** (eg, from dietary intake or polluted/high altitude H2O) **Benzocaine** (a local anaesthetic in the "esters" group) **by cause poisoning by oxidizing Fe2+ to Fe3+.** **note: nitrited used to INDUCE Methemoglobinemia in cases of CN poisoning**
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**Rugal hypertrophy IN BODY** not antrum looks like **brain gyri** lost of **mucus prod** due to HYPERTROPHY OF FOVEOLAR PITS lots of protein LOSS (labs = **hypoalbuminemia-**-\> edema) **DECREASED acid prod (hypochorlydria)** only ONE thing this can be!
**Menetrier Disease!** theres **IDIOPATHIC gastric HYPERplasia of the MUCOSA**
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how does myelin affect the **time constant** and **length constant?** **explain in your own words what these two concepts mean.** how do the time and length constant (unfavorable) change, with decreased myelination?
**Time constant** is **HOW LONG it takes** to reach a new membrane potentional, the shorter amont of time, the faster the axonal conduction [**MYELIN DECREASED THE TIME CONSTANT** (this is favorable) **Length constant** is **HOW FAR along an axon** an electrical impulse can propogate [**MYELIN INCREASED THE LENGTH CONSTANT** (this is favorable) opposite is the case if there is demyelination!
418
Anti-Scl-70 is also known as and is seen in what diseae?
Anti-Scl-70 aka: **(anti-DNA topoisomerase I)** **Scleroderma (diffuse)**
419
whats the exact role of ATP in the cardiac and skeletal muscle cycle as it relates to mysosin?
**ATP** is needed for the **MYOSIN** head (crossbridge) to **DETACH from ACTIN!!!** if you **dont have ATP** you get r**igor mortis (just stiff)..**. **BC THE CROSSBRIDGE PERSISTS!!!** **KNOW THAT PIC!**
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whats the key to pathogenicity in organisms that make little toxin, give an example.
**Invasion** is **the key** to pathogenicity; organisms that produce little toxin can cause disease due to invasion **shigella** * **gram ⊝ rods, non-lactose fermenters, oxidase ⊝** * **can invade the GI tract via M cells of Peyer patches.**
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What disease/bug...WHERE WOULD A PERSON INFECTED BY THIS BUG MOST LIKELY RESIDE (EPI is very important)? fever hemolytic anemia splenomegaly intra-erythrocytic inclusions "maltese cross" IXODES TICK is the vector (what other 2 diseases use Ixodes as the vector)
Protozoa: BABESIA causes BABESIOSIS NORTHEAST USA same as LYME DISEASE AND ANAPLASMOSIS (both use ixodes)
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which asmtha drug is **Especially good for aspirin-induced asthma.**
**Montelukast, zafrlukast**—block **leukotriene receptors** (CysLT1).
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LACKS CELL WALL HIGH TITERS OF COLD AGGLUTININS (IgM) which are Abs that are produced in response to this bug--\> can agglutiniat or lyse RBCs [**whats the pathogenic mechanism behind this**?] cell membrane contains STEROL for STABILITY X ray looks worse than patient **name that bug** & **what population are they mostly seen in**?
Cold agglutinins are Abs directed against **Antigens** in the cell membrane of **MYCOPLASMA PNEMONIAE (the bug!) that just so happen to be the same as the Antigens present on the surface of human RBCs** **seen in military recruits and prisons**
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Wilms tumor, early-onset nephrotic syndrome, male pseudohermaphroditism (WT1 mutation)
**Denys-Drash disease** **one of the many associations with WT1 mutation**
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whats the diff in an EEG in some with DELERIUM vs someone with DEMENTIA which one is usually reversible?
**DELIRIUM: diffuse *_SLOWING OF EEG_*** \*reversible **DEMENTIA: *_NORRRMAL!_*** \*usually not reversible
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pick one Cimetidine Ondansetron Omeprazole Sucralfate Metoclopramide
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Adults present with cutaneous lesions; especially affects populations in Japan, West Africa, and the Caribbean. Lytic bone lesions, hypercalcemia.
Adult T-cell lymphoma Caused by HTLV (associated with IV drug abuse)
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**low voltage ECG + electrical alternans** is a phrase that indicates what pathological heart state? whats special about the Diastolic pressures? whats special about the systolic Blood pressure (looking for a special phenomenon here)..what other 4 disease is this phenomenon associated with?
**Cardiac tamponade** **Equilibration** of **diastolic pressures in all 4 chambers.** **Pulsus paradoxus** DEC in amplitude of **systolic BP by \> 10 mm Hg** during inspiration. Seen in cardiac tamponade, a**sthma, obstructive sleep apnea, pericarditis, croup.**
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whats the nutcracker effect? what condition can it lead to which does not transilluminate?
aka: nutcracker phenomenon, **renal vein entrapment syndrome,** or **mesoaortic compression of the left renal vein.** **It results most commonly from the compression of the left renal vein between the abdominal aorta (AA) and superior mesenteric artery (SMA),** **associated with hematuria (which can lead to anemia)[3] and abdominal pain (classically left flank or pelvic pain).[4]**
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diff btw the two following IMMATURE defense mechanisms **Fixation** vs **R****egression**
**Fixation:** is being an adult who acts like a kid (having the maturity level of a child as a grown ass adult); adult who still plays video games every day at the age of 48. vs **regression:** *involuntarily* reverting back to a younger (could be due to an illness, stress, new sibling); eg: a kid who WAS potty trained all the sudden beginning to wet his bed.
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what type of genetic principle should be suspected if a genetic mutation is identified in offspring BUT NOT THE PARENTS?
GERMLINE MOSAICISM!!!!
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mg2+ excess and def. what happens
**LOW Mg2+** Hypocalcemia with tetany (acquired hypoparathyroidism due to impaired PTH secretion and resistance in target tissue) **Tachycardia** **EXCESS mg2+** Neuromuscular depression (depressed deep tendon reflexes, muscle weakness) **Bradycardia**
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why do ace-I pts have an inc in Cr initially? when is this an issue?
bc abolishing the eff art vasocontriction effect leads to a **DEC Filtration fraction with dec GFR.** \*usually normalizes in a few weeks this is **only an issue in** ppl who have DEC GFR states to begin with like **bilat RAS** **decomp HF** **chronic kidney disease** **volume depletion** but actually ACE-I are renoprotective for diabetic pts bc they reduce the hyperfiltration injury
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Risk factors for CERVICAL **Dysplasia and carcinoma in situ** **4 of them!**
Risk factors: multiple sexual partners (#1), smoking, starting sexual intercourse at young age, HIV infection.
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which bugs produce HYALURONIDASE (an enzyme used to digest extracellular ground substance and enhance their ability to spread) 3 bugs (all gram +ve)
Staph Group A strep (Pyogene) Clostridium difficile
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precocious puberty in males is seen with this childhood brain tumor bc it makes (β-hCG production).
Pinealoma
437
what receptor does CMV use? how about Rabies how about Rhino virus (i cam)
Integrein (hepara sulfate) what receptor does CMV use? **Integrein (hepara sulfate)** how about Rabies **Nicotininc AchR** how about Rhino virus **(i cam)**
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They are used in the treatment of chronic diarrhea due to bile acid malabsorption. Chronic diarrhea may be caused by excess bile salts entering the colon rather than being absorbed at the end of the small intestine (the ileum). This condition of bile acid malabsorption occurs after surgery to the ileum, in Crohn's disease, with a number of other gastrointestinal causes, or is commonly a primary, idiopathic condition. The SeHCAT test can be used for diagnosis. Bile salt diarrhea can also be a side-effect of gallbladder removal. Bile acid sequestrants are the principal therapy for bile acid-induced diarrhea.[6][7] Cholestyramine, colestipol and colesevelam have all been used. Doses may not need to be as high as those previously used for hyperlipidemia. Many patients find them hard to tolerate, as although the diarrhea may improve, bloating and abdominal pain can worsen.[8][9]
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whats the main mediator in DIC during pregnancy, for example, duing Placenta Abruption due to one of the RF's like HTN? (hint: its found in high concetration in placental trophoblast)
TISSUE FACTOR (aka: THROMBOPLASTIN)
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if a middle aged woman has **INC ALP** levels, you should check for levels of WHAT in the blood to see the origin of the issue? pick one. **unconj BR** **GGT** **PT** **BUN**
**GGT!!!** bc ALP is found mostly in BONE and LIVER so GGT can tell you if the issue is of BONE OR LIVER origin BC GGT is NOT found in the BONE!!!
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**Therapeutic supplemental O2** can result in what 3 complications (mainly given to premature infants) ## Footnote **(RIB)**
**1. Retinopathy of prematurity,** **2. Intraventricular hemorrhage** **3. Bronchopulmonary dysplasia** **(RIB)**
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what is the DOC for URGE incontinence and what is urge incontinence? there are three types of incontinence remember.
**MUSCARINIC ANTAG!** **Oxybutynin (first and foremost),** solifenacin, tolterodine (careful not to use Muscus antag in someone with angle closure glaucoma **they reduce bladder spasms and urge urinary** **incontinence (overactive bladder).** **Overactive bladder (detrusor instability)--\>leak with urge to void immediately. T** **reatment: Kegel exercises, bladder training (timed voiding, distraction or relaxation techniques), antimuscarinics (eg, oxybutynin).**
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in a person who just got a tranfusion, what must you absolutely use to diff between "febrile NON hemolytic transf rxn" and acute HEMOlytic transf rxn?
**HEMOGLOBINURIA** (PEE ALLS THE SUDDEN IS DARK)---bc of hemolysis! note that the **hemolytic type** is much **more severe.** can lead to DIC; its also strats within MINUTES! fast! **intravasc hemolysi**s if ABO blood group incompatibiliut and **extravasc hemolysis** if host ab rxn against foreign ag on donor rbcs
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INCREASED risk associated with **natalizumab, rituximab.** Demyelination of CNS due to destruction of oligodendrocytes. Seen in 2–4% of AIDS patients diagnosis?
**Progressive multifocal leukoencephalopathy (PML)** reactivation of latent JC virus infection).---POLYOMA virus
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in the myofibril: a. which bands decrease in length upon contraction b. which dont change in length everrrr c. on EM, are the thin actin filaments dark or light, hows about the thick myosin filaments? d. H band straddles what e. whats in the middle of the I band? f. what filaments are exclusive to the H band, hows about the I band? what about the A band?
a. H and I b. A! c. thin (actin) are LIGHT, thick (myosin) are DARKER d. the M line! e. Z line (darkest line on EM!) f. thin for H, thick for I and A has both think and thick (H band is included inside the A band).
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HIV +VE PT CD\<50 hsmegaly inc ALP inc LDH CLEAR chest xray bug grows optimallly at 41 degrees what infection and what bug!
Mycobac Avium Complex look at that low ass CD count it grows in the special temp CLEARRR chest xray it targets the RES system! give AZITHROMYCIN for prophylaxis NOT isoniazid, it wont work!
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if a vingnette tell you someone has 1. persistent diarrhea 2. ulcers BEYOND DUODENAL BULB AND IN JEJUNUM 3. refracotry to therapy whats the diag
**ZE!!!!!** just bc it says diarrhea dont get tricked. key is they can happen beyond the duodenum and meds wont help. will do test to see if they are associated with MEN1 (often times are!), so check prolactin and Ca2+) and also so the secretin test
448
False-positive results on VDRL with:
False-positive results on VDRL with: **Vi**ral infection (eg, **EBV, hepatitis)** * *D**rugs * *R**heumatic fever **L**upus and **l**eprosy
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pts who take ACE Inhibitors develop that cough bc ACE is normally used to breakdown Bradykinin but also what other substrate?
Substance P
450
for **POST OP** nausea, what can you give? - ondansetron - metoclopromide - first gen H1 blockers (diphenhydramine, Meclizine, -Cyclizine) - dronabinol
5-HT3 antagonist; DEC vagal stimulation. Powerful **central-acting antiemetic.** **Ondansetron !!!!! is the "best choice"** ---can also be used for chemo-induced nausea **-metoclopromide (prokinetic)** is more for **Diabetic and postsurgery gastroparesis (5HT4 agonist) and D2 antagonist** and is an *antiemetic* **HIGH doses---** IT INC resting tone, contractility, LES tone, motility. Does not influence colon transport time. ***watch out for EPS sxs*-** **first gen H1 blockers** (diphenhydramine, Meclizine, -Cyclizine) is for **MOTION SICKNESS** (so vesibular nausea)--these cross BBB. also scolpalamine would be wrong for the same reason **dronabinol;** NO! its for ppl on **chemo** with nausea! Pharmaceutical form is **dronabinol** (tetrahydrocannabinol isomer): used as **antiemetic (chemotherapy**) and **appetite stimulant (in AIDS).**
451
what would the labs be in someeone with RA?
HLA-DR4+ RFs: smoking, silica exposure. * **_⊕ rheumatoid factor_** (**anti-IgG antibody**; more specifically its the M that tarts the G); **IgM antibody that targets *IgG Fc* region); in 80%),** \*note Fc is the region on the antibody that binds Macs and Complement * **anti-cyclic citrullinated peptide antibody-** anti-CCP *(more specific)!* *RF is not diagnostic bc is seen in a bunch of other things too.*
452
what drugs cauase Aplastic anemia? mnemonic is: **C**an’t **M**ake **N**ew **B**lood **C**ells **P**roperly
Carbamazepine, Methimazole, NSAIDs, Benzene, Chloramphenicol (dose depedent=*anemia* & dose independent *APLASTIC anemia*)-Limited use owing to toxicities but often still used in developing countries because of low cost. Propylthiouracil **C**an’t **M**ake **N**ew **B**lood **C**ells **P**roperly also Radiation, alkylating agents, and antimetabolites
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* Recombinant **PTH analog** given subcutaneously daily. * INCREASES osteoblastic activity. * Treats: **Osteoporosis.** * MOA: *Causes bone growth* **compared to** antiresorptive therapies (eg, bisphosphonates: which are-Pyrophosphate analogs; bind hydroxyapatite in bone, *inhibiting osteoclast activity).*
**Teriparatide**
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A pt returns to your clinic fr. **Latin America** with signs **of Asthma..wheezing non prod cough.** But a stool sample shows a round curved worm (slide is given). YOUR ATTENDING TELLS YOU THIS IS **THE MOST COMMON HELMINTH INFECTION IN THE WORLD...including US**! You are looking at what and will treat with what? AND also seen is what is MOA of the drugs? BONUS, you must know.
YOU are looking at **ASCARIASIS.** So common. Treat with Mebendazole (WHICH WORKS BY BLOCKING GLUCOSE UPTAKE). gives you Loeffler syndrome Early phase (The early phase coincides with larval tissue-migration. Typically, 4-16 days after egg ingestion.) The main symptoms include the following: **-fever** **-cough** **-wheeze** then later Intestinal infection with possible **obstruction at ileocecal valve** This is FECAL ORAL SPREAD. **pork products**
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**bifid carotid pulse with a brisk upstroke** (spike and dome) is describing what condition?
**HOCM!!!!!** **lv outflow tract obstruction at systole**
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DERIVATIVE---\> thyroid follicular cells DERIVATIVE --\> parafollicular (C) cells of thyroid
ENDODERM---\> thyroid follicular cells NEURAL CREST---\> parafollicular (C) cells of thyroid
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when a patient with turner syndrome is born with TWO genotypes (eg: 46,XO/46,XX), what genetic phenomenon is this due to? what two other disease is this phenomenon associated wiht?
**_MOSAICISM_** **"Mosaic Turner Syndrome"** Mosaicism is *also seen in:* Most commonly: **MC CUNE ALBRIGHT SYNDROME** also: in 1% of **Down syndrome** cases
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exposure to moldy hay or contaminated compost full of **thermophilic actinomycetes** is classically known to cause what lung pathology **Clues in the history include • Symptom onset after moving to a new job or home** **• A hot tub, a sauna, a swimming pool, or other sources of standing water or water damage in the home or regular exposure to them elsewhere** **• Having birds as pets • Exacerbation and relief of symptoms in and away from specific settings**
HYPERSENSITIVITY PNEUMONITIS (very much occupational) (FARMERS LUNG) TYPE 3 AND 4 HS RXN - dont need to be a smoker - dont need to have a history of asthma or allergies pt may have **• Recurring atypical pneumonias** The most typical HRCT finding is the presence of profuse, **poorly defined centrilobular micronodules.** The disorder seems to represent a **type IV hypersensitivity reaction**, in which **repeated exposure to antigen** in genetically susceptible people leads to **acute neutrophilic and mononuclear alveolitis,** followed by **interstitial lymphocytic infiltration and granulomatous reaction.** **Fibrosis with bronchiolar obliteration occurs with continued** exposure. **Acute disease** occurs in **previously sensitized people** with acute **high-level antigen exposure** and manifests as **fever, chills, cough, bilateral vice-like chest tightness** (as can occur in asthma), and dyspnea **4 to 8 h after exposure.** Anorexia, nausea, and vomiting may also b e present. Physical examination shows tachypnea, diffuse fine-to-medium inspiratory crackles, and, ***_in almost all cases, absence of wheezing._*** Chronic disease occurs in people with chronic low-level antigen exposure (such as owners of birds) and manifests as onset over months to years of exertional dyspnea, productive cough, fatigue, and weight loss. There are few physical findings; clubbing uncommonly occurs and fever is absent. In advanced cases, pulmonary fibrosis causes symptoms and signs of right heart failure, respiratory failure, or both
460
RARE **VASCULAR** TUMOR EXPRESSES **CD31 (AKA: PECAM-1)** WHAT IS IT, WHATS IT DUE TO CHARACTERISTICALLY??? WHY IS PECAM-1 FAMIILAR?
**ANGIOSARCOMA (OF LIVER)** DUE TO **CARCINOGENS** **1. VINYL CHLORIDE** **2. ARSENIC** **3. THOROTRAST!** **PE CAM IS EXPRESSED ON VASC ENDOTHELIUM** (BUT ALSO ON THE WBC) DURING **"TRANSMIGRATION OR DIAPEDIS OF WBC THROUGH THE ENDOTHELIUM!!!**
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how are levels of Prolactin affected by TRH?
TRH prolactin **INCREASES secretion!** for (eg, in 1° or 2° **hypo**thyroidism) where TRK is increased! also: **Dopamine antagonists** (eg, most **antipsychotics**) and **estrogens** (eg, OCPs, pregnancy) **stimulate prolactin secretion.**
462
a Cerebrovascular accident (transient ischemic attack/**stroke**) in the setting of known venous thromboembolic disease (Eg: lower extremeity D**V**T) is suspicious of\_\_\_\_\_\_\_
Paradoxical embolsim (**venous** thromboemboli that enter **systemic** arterial circulation), eg: seen in **Patent foramen ovale**—caused by failure of septum **primum** and septum **secundum** to fuse after birth; most are left untreated. if untreated can have this complication.
463
WHAT ARE THE B CELLS LIKE IN A BABY WHOSE MOM HAD UNCONTROLLED maternal diabetes
baby has BETA CELL HYPERPLASIA!!!! MAKES A TON OF INSULIN WHEN ITS BORN, MOM HIGH GLUCOSE NO LONGER BEING TRANSPLACENTALLY TRANSFERRED SO BABY IS PROFOUNDLY HYPOGLYCEMIS AND FAT (MACROSOMIA) NOTE: INSULIN DOESNT CROSS PLACENTA! SO DONT PICK AN ANSWER THAT HAS TO DO WITH INSULIN FROM THE MOM IN THE BABY IN UTERO
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**Autosomal dominant** disorder most common in **Asian males.** **CHARACTERISTIC**: ECG pattern of **pseudo-right bundle branch block** and **ST elevations in V1-V**3. **Inc risk of ventricular tachyarrhythmias** **and SCD**. Prevent SCD with implantable cardioverter-de brillator (ICD). what disease?
Brugada syndrome
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what are the two (thrombopoietin receptor agonists) used to treat thrombocytopenia?
**Romiplostim** **eltrombopag**
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**Primary biliary Cirrhosis (aka: Primary biliary cholangitis-Anti-mitochondrial antibody ⊕)** is has a *similar histopathological presentation as* which other immunologically related disorder?
GRAFT VS HOST DISEASE ## Footnote both are characterized by: **lymphocytic infiltrate + granulomas destruction of intralobular (intrahepatic) bile ducts.**
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**Opsoclonus- myoclonus ataxia syndrome** “Dancing **eyes,** dancing **feet”** is associated with what two cancers different in kids vs adults
**Neuroblastoma (children),** **small cell lung cancer (adults)**
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the resovoir for Mycobacterium LEPRAE **in the US (not overseas)** is WHAT ANIMAL
a freakin **armadillo**!
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**lens subluxation** **marfanoid habitus** **increased risk for thrombosis** in small and large vessels (most common cause of death) **AR disorder** whats the disease, enzyme deficiency (2 enzymes) and cofactor necessary for these?
**HOMOCYSTINURIA** (MOST COMMON: **CYSTATHIONINE SYNTHASE DEFICIENCY** ---needs **vit B6** (PLP=pyridoxal phosphate is the active form of Vit B6) ALSO: **METHIONINE SYNTHASE DEFICIENCY-**--needs **vit B12**
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**Pale (anemic) infarcts** occur in what **THREE organs**
Pale (anemic) infarcts occur in **solid organs** with a **single (end-arterial) blood supply**, such as **heart, kidney, and spleen.**
473
match the main AE with the DRUG Class or name {diabetes drug} 1. **Hypoglycemia**, lipodystrophy, rare hypersensitivity reactions. 2. Weight gain, **Fluid retention, edema**. **Hepatotoxicity (requires hepatotox monitoring)**, HF (so CI in pts with class III/IV) HF, risk of fractures. 3. Pancreatitis (**ps**: which NRTI causes pancreatitis as a main AE?) **pss**: what **enzyme** deactivates these anti-diabetes drugs? 4. **Mild urinary or respiratory infections** (ps: does this drug have any effect on weight?) 5. Just **GI sxs (diarrhea and flatulence)** 6. **Glucosuria (**big hint about its MOA**)**, UTIs, **vaginal yeast infections**, hyperkalemia, dehydration (**orthostatic hypotension**). 7. what are the two drug classes that act to stimulate release of insulin by **closing the K+ channels** in pancreatic beta cells allowing for depol and subsequent **release of Insulin** but act at **different sites** on those K channels? ps: whats an AE they share? 8. GI upset; most serious adverse effect is * *lactic acidosis** (thus **contraindicated in renal insuf ciency**). whats this drugs MOA?
1. **Insulin preps** (rapid, short, intermediate and long acting) 2. _Glitazones_ Pio**glitazone** (improves HDL), Rosi**glitazone** **MOA:** Binds to **PPAR-γ nuclear transcription regulator.** 3. _GLP-1 analogs_ * *-Exenatide** - Liraglu**tide** (sc injection) answer to **PS**: *DIDANOSINE (NRTI)* answer to **PSS**: *DPP-4 enzyme* 4. _DPP-4 inhibitors_ Lina**gliptin**, saxa**gliptin**, sita**gliptin** **MOA**: inhibit *DPP-4 enzyme! (the one we just talked about up there!)* 5. α-glucosidase inhibitors Acarbose, miglitol **MOA:** ***delay carb hydrolysis and glucose absorption** by a*cting at the intestinal brush border & **Inhibitig** ***alpha-glucosidases**--\> decreased abs of disaccharides* 6. _Sodium-glucose co-transporter 2_ **_(SGLT-2) inhibitors_** Cana**gliflozin**, dapa**gliflozin,** empa**gliflozin** **MOA:** decrease reabsorption of glucose in PCT--\> promote an osmotic diuresis: **DECREASE:** vL, BP, Phosphate and **INCREASE:** K+, Mg2+, Cr 7. _Sulfonylureas_ **First generation:** chlorprop**amide**, tolbut**amide** **Second generation**: **glip**iz**ide**, **glime**pir**ide**, **gly**bur**ide** **AND** they are called **"Insulin Secretagogues"** _Meglitinides_ Nate**glinide**, repa**glinide** **PS:** both are Contraindicated in renal failure, both INCrease weight and both can cause hypoglycemia 8. _Biguanides_ **Metformin** **MOA: reduces gene expression of gluconeogenic enzymes;** +activates AMPK and decreases insulin resistance and decreases GNG
474
how do you differentitate btw SLE and DILE (drug induced SLE) as far as immunological markers are concerned?
DILE = **ANTI HISTONE ABS** IN 95% OF CASES of DILE SLE = **ANTI dsDNA abs** in SLE but RARELY in DILE
475
Features of SLE, systemic sclerosis, and/or polymyositis. Associated with **anti-U1 RNP antibodies (speckled ANA).**
**Mixed connective tissue disease**
476
pt has good ol **kidney stones** takes no drugs no other sxs what are his blood and urine levels of Ca2+ like?
NORMAL CALCEMIC!!!!! **HYPER**CALCI**URIA** **unless pt has sxs of something that inc ca+ in blood** **like Hyperpara, sarcoidosis, hyperacidemia, malignancy they will have NORMAL ca2+ levels in blood!!!!**
477
More common in **firstborn males**; associated with **exposure to macrolides**. Results in **hypokalemic hypochloremic metabolic alkalosis**
Hypertrophic pyloric stenosis
478
BORDETELLA PERTUSSIS DOC?
MACROLIDES AZITH CLARITH ERYTH
479
ddwhen in a womans cycle is estrogen about the highest and progestrone is low? day: 1, 6, 12, 18 or 26?
day 12!!!!
480
“seal-like” ***_barking cough_*** and inspiratory stridor causes WHAT BY WHAT BUG.
**Caused by parainfuenza viruses (paramyxovirus).** Results in a “seal-like” barking cough and inspiratory stridor. Narrowing of upper trachea and subglottis leads to characteristic **steeple sign** on x-ray . Complication: Severe croup can result in **pulsus paradoxus** (2° to upper airway obstruction)
481
what is the most important facto**r affecting BMR**
**Lean body mass** most important factor affecting BMR * *Key factors a ecting BMR** * *(1) Body mass**—the greater the amount of lean body tissue, the higher the BMR **• Lean body tissue** is more metabolically active than fat, so the way to increase BMR to the maximal rate is to make endurance and strength-building activities a daily habit. (**2) Gender—**men’s lean body mass greater than women’s * *(3) Hypothyroidism**—BMR is lower (hypometabolic) * *(4) Hyperthyroidism**—BMR is higher (hypermetabolic)
482
in which benign liver tumor is it CONTRAINDICATED TO DO A BIOPSY DUE TO RISK OF HEMORRHAGE?
CAVERNOUS HEMANGIOMA!! BENIGN COMMON AGE 30-50
483
**confusion, ophthalmoplegia, ataxia** (classic triad) + confabulation, personality change, memory loss (permanent). Damage to medial dorsal nucleus of thalamus, mammillary bodies. what disease process is being described
Wernicke-Korsako syndrome
484
NOCTURNAL PAIN in bone relieved by ASPIRIN, What bone pathology what characteristic feature does it have?
OSTEOID OSTEOMA (benign bone tumor that arises from **osteoblasts**) HAS A **CENTRAL NIDUS** (Radiographs in osteoid osteoma typically show a round lucency, containing a **dense sclerotic** central nidus (the characteristic lesion in this kind of tumor), surrounded by sclerotic bone. The nidus is seldom larger than 1.5 cm
485
WHAT ARE **3 DRUGS** ASSOCIATED WITH **Erythema multiforme** (NOTE: MAIN ASSOCIATION WITH ERYTH MULTIFORMM IS **HSV** BUT **ALSO** Associated with infections (eg, **Mycoplasma pneumoniae**
phenytoin, sulfa drugs, β-lactams,
486
this organ has foregut blood supply but arises from mesoderm in the dorsal mesentary of the stomach
SPLEEN supplied by celiac trunck---\>splenic artery (foregut structure)
487
fever and **hemolytic anemia;** predominantly in **northeastern United States;** **asplenia** risk of severe disease **Ixodes tick** (same as Borrelia burgdorferi of Lyme disease; may often coinfect humans) **Atovaquone + azithromycin to treat**
Babesiosis !!!!!! Blood smear: ring form **“Maltese cross”**
488
being of native american origin is associated with what type of gallstones? cholesterol or pigmented is TPM (total parenteral nutrition associated with chol or pigment gallstones?
native america = RF for CHOLESTEROL TPN = RF for pigment
489
what childhood brain tumor is being descsribed Can cause **Parinaud syndrome** (compression of tectum vertical gaze palsy); **obstructive hydrocephalus** (compression of cerebral aqueduct); **precocious puberty in males** (β-hCG production). **Histologically similar** to **germ cell tumor**s (eg, **testicular seminoma).**
**Pinealoma** ## Footnote pineal gland sits right above the **SUPERIOR COLLICULI** which is why **PARINAUD syndrome** (which is: paralysis of conjugate vertical gaze due to lesion in superior colliculi (eg, stroke, hydrocephalus, pinealoma).
490
whats the only systemic mycoses that is NOT a yeast in the heat (its dimorphic, but takes on a different shape than all the rest of the systemic mycoses) name the bug and what it becomes at 37 degrees instead of a mold
COCCIDIOIDOMYCOSIS IS A **SPHERULE at 37 degrees in tissue**
491
congenital heart defect caused by ANTEROSUPERIOR DISPLACEMENT/DISPLACEMENT OF **THE INFUNDIBULAR SEPTUM**
tetrology of fallot squatting helps to INCREASE SVR (inc afterload) and helps dec the right--\> left shunt
492
what are the 9 main etiologies of **Acute respiratory distress syndrome** **SPPARTAS**:
**SPPARTAS**: **S**epsis, **P**ancreatitis, **P**neumonia, **A**spiration, u**R**emia, **T**rauma, **A**mniotic **F**luid embolism, **S**hock- -\> Endothelial damage increased alveolar capillary permeability--\> protein-rich leakage into alveoli--\> **diffuse alveolar damage (DAD)** and **_noncardiogenic_ pulmonary edema**
493
bacterial vaginosis in pregnancy predisposes to what?
premature rupture of membranes
494
Autoantibodies to **presynaptic Ca2+ channel of the neuromuscular junction** --\> **ACh release** **how do you diff this from its similar counterpart?**
**Lambert-Eaton syndrome** **Arises as a paraneoplastic syndrome,** most commonly due to **small cell carcinoma of the lung (vs MG:** Associated with **thymic hyperplasia or thymoma; thymectomy improves** **symptoms.** **Lambert eaton:** Leads to **impaired acetylcholine release** **1. Firing of *presynaptic calcium channels* is required for acetylcholine release.** **Clinical features** **Proximal muscle weakness that _improves with use;_ *eyes are usually spared (vs MG shows: Ptosis, diplopia)*** **\*\*\*\*\*\*\*\*\*\*\*\*\*\*\*Anticholinesterase agents do not improve symptoms (unlike MG)** **Resolves with resection of the cancer**
495
This is an infection commonly seen in **immunocompromised** (HIV, TRANSLANT (*think CMV*), immunosuppresant drugs, etc) presentation on esophagastroduodenaoscopy: are **linear shallow ulcers** pt will present with either **dysphagia** (diffulculty swallowing) or **odynophagia** (pain with swallowing) + fever + burning chest pain **Shallow linear ulcers** are seen on endoscopy. what will be seen on biopsy of esophagus? what does this pt have?
**CMV esophagitis!** **Basolphiiic INTRANUCLEAR inclusions** CMV--\> shallow **linear** ulcers if--\> Candida: white pseudomembrane if HSV-1--\> punched-out ulcers
496
which lipid lowering agent is the ONLY one to INC TAGS (slightly but still matters) as a consequence of their MOA?
**Bile acid resins** - choestyramine - colestipol - colesevelam since they stop intestinal absorption of bile acids, the liver takes up cholesterol from blood to make more bile acids but in turn also increases hepatic prod of TAGS:(
497
during epithelial repair of the small intestine, where is the most active cell division occuring?
New epithelial cells are generated in the ***_base of the crypt_*** and migrate and differentiate during their journey along the villus until they are shed at the extrusion zone at the villus tip. mitosis at base of the crypt furthest from the lumen and then the daughter cells migrate up the the tips (villI)
498
499
whats the epithelium in the ovary? endocervix (internal os) ectocervix (external os)
simple cuboidal simple columnar NK stratified squamous epi
500
Fibrils composed of **β2-microglobulin** in patients with **ESRD and/or on long-term dialysis.** May present as carpal tunnel syndrome. this is how amyloidis related to WHAT procedure presents
**Dialysis-related Amyloidosis**
501
if an elderly person seems to have dementia what should you screen for?
screen for depression hypothyroidism (TSH) b12 these can present like dementia (PSEUDODEMENTIA)
502
most common cause of death in PNH?
venous thrombosis!!!! dont be fooled. if you know the pathogenesis you know that the issue is an ACQUIRED mutation in a HEMATOPOEITIC STEM CELL THAT MAKES CELLS FROM ALLLLLLL LINEAGES (RBC, PLTS, WBC (GRANULOCLYTES), THAT LACK THE GPI ANCHORING MOLECULE (BC PIG-A GENE SOMATIC MUTATION) SO DAF (CD55) AND CD59 (MIRL) CANT STOP COMPLEMENT FROM LYSING THESE CELLS BC THEY USUALLY PROTECT THESE CELLS FROM LYSIS PLTS ARE INCLUDED IN THIS BUNCH SO ALL THE LYSED PLTS RELEASE THEIR CYTOPLASMIC GRANULES AND ACTIVATE COAG CASCADE! **VEIN THROMBOSIS!!!!!!**
503
Renal cell carcinoma, hepatocellular carcinoma, hemangioblastoma, pheochromocytoma, leiomyoma all can lead to what COMMON paraneoplastic presentation and produce what
these conditions can produce INC EP--\> **"innapropriate/pathologic"** ## Footnote **Reactive** **SECONDARY Polycythemia**
504
hypertrophy vs hyperplasia
**HYPERTROPHY** INC IN in **size of cells.** **HYPERPLASIA**: INC in number of cells. May be a risk factor for future malignancy (eg, endometrial hyperplasia) but not considered premalignant.
505
which one has thrombocytpenia due to having **dec # of and smalled plt..** **HYPER IGE (job)-autosomal dom** **OR** **WISKOTT ALDRICH SYND (XR)**
**WISKOTT ALDRICH SYND (XR)!!!!** WA**T**ER!!!! **W**iskott-**A**ldrich: **T**hrombocytopenia, bczema, **R**ecurrent infections. \*risk of autoimmune disease and malignancy. **HYPER IGE is: FATED:** coarse Facies, cold (nonin amed) staphylococcal Abscesses, retained primary **T**eeth, IgE, Dermatologic problems (eczema).
506
when **clostridium dificile,** secretes an endotoxin that causes watery diarrhea (DUE TO WATER LOSS FROM TISSUE TO THE INTESTINAL LUMEN) it does so by binding to AND *INTERFERING WITH* WHICH CELL JUNCTION **PROTEINS** that correspond to WHICH TYPE OF **CELL JUNCTION TYPE**?
IT **INTERFERES WITH _*CLAUDIN AND OCCLUDIN* PROTEINS_** WHICH MAKE OF ***_tight junctions_*** WHICH ARE IN CHARGE OF **ACTING AS A PARACELLULAR BARRIER TO WATER AND SOLUTES** **\*LOOK AT CELL JUNCTIONS SHEET IN IPAD FOR DETAILS**
507
**Severe pyogenic i**nfections early in life; ***_opportunistic infection_*** with **Pneumocystis, Cryptosporidium, CMV, Histoplasma, Bartonella,** normal NUMBER OF B CELLS what immune def is this?
**Hyper-IgM syndrome** Most commonly due to defective **CD40L on Th cells** **no: IgG,** IgA, IgE. no opsonization! no class switch
508
a medical condition characterized by the **inability to control facial movements** (such as chewing and speaking) and caused by a variety of neurological disorders. Patients experience **difficulty chewing and swallowing,** have **increased reflexes and spasticity in tongue and the bulbar region**, and demonstrate **slurred speech** *_(which is often the initial presentation of the disorder),_* sometimes also demonstrating uncontrolled emotional outbursts. The condition is usually caused by the **damage (bilateral degeneration) to the neurons of the brain stem, specifically to the corticobulbar tract** (upper motor neuron tract to cranial nerve motor nuclei).
Pseudobulbar palsy
509
what TB med is highly associated with causing a type of microcytic anemia that is characterized by having an accumulation of Fe2+ in the mitonchondria? hint: if this condition is congenital (not acquired like in the case of this med example, it is due to an ALA synthase deficiency) whats the condition and what is the pathogenesis of this drug interaction?
ISONIAZID--\> VIt B6 deficiency--\> SIDEROBLASTIC ANEMIA VIt B6 is needed as co factor for 1st of Protoporphyrin synthesis for enzyme ALA synthase--\> no Protoporphyrin--\> iron accumulates, no heme made----\> No Hb made--\> microcytic anemia
510
can you treat cutaneous fungi with **nystatin?**
NOOOOO!!!!!! you use it for Swish and swallow” for **oral candidiasis (thrush**); topical for **diaper rash (CUTANEOUS CANDIDA!)** or **vaginal candidiasis.** **not skin fungi like dermatophytes (you use**
511
what are LINES OF ZAHN made of? list the 2 ingredients do they tell you that the thrombi formed after OR before death?
**platelts and fibrin!!!!!!!!!!!!!** tells you it fomed **BEFORE DEATH (its a pre-mortem)** thrombus.
512
Prostaglandins produced by the intrauterine tissues of both mother and fetus (myometrium, decidua, placenta, chorion, and amnion) are involved with all of the physiologies of parturition (membrane rupture, cervical dilatation, myometrial contractility, placental separation, and uterine involution). For parturition to occur, however, the intrauterine tissues need to first be activated to prepare for the work of labor, then stimulated to initiate labor. Prostaglandins normally are considered to be stimulators of the physiologies of labor. This review presents evidence that one prostaglandin, PGF2alpha, and its receptor, FP, are also activators, especially of the decidua. Stimulated by cytokines, the decidual synthesis of PGF2alpha and the expression of FP lead to increased matrix metalloproteinase activity, further enhancement of cytokine activity, increased decidual oxytocin and oxytocin receptor expression, decreased progesterone responsiveness, and possibly, enhanced expression of vascular endothelial growth factor. These collective actions prepare the decidua for its role in parturition.
513
classic symptom of **erythromelalgia** (*severe, burning pain and red-blue coloration*) due to episodic blood clots in vessels of the extremities is commonly seen in what two hematological disorders? but one its known for and the other i can happen sometimes
**Polycythemia vera (main one)** **can be seen in: Essential thrombocythemia too**
514
what is a very common complication of mesothelioma? be as **SPECIFIC** as possible? use LOGIC based on WHERE mesothelioma affects what does it look like on xray and CT? just as a bonus: what characteristic histo feature will be seen?
PULMONARY EFFUSION but a **HEMORRHAGIC (BLOOOOD!!!!)** **exudative** (not transudative) **UNILATERAL** usually **large** **PULMONARY EFFUSION** (on x ray, the two sides of the diaphragm will NOT BE EVEN bc of the fluid) and on CT there will be GRAY amonst the BLACK which is AIR (should be all AIR!) **Bonus: PSAMMOMA BODY!**
515
small cell lung ca is associated with a NMJ disease whose pathogenesis is what. how are levels of Ach affected?
**AUTOANTIBODIES to *PRE*-synaptic _Ca2+ CHANNEL_-**--\> **decreased Ach release!**
516
in tissue ischemia what ions are outside and what ions are inside?
K is the only freakin ion found OUTSIDE in the extracellular space bc ATPase doesnt work so it leaks out NA+, Ca2+, H2O, Hco3-, Cl- all come INTO THE CELL CYTOPLASM
517
why can yo NOT take tetracyclines with the following? 1. milk (Ca2+) 2. antacids (Ca2+ or Mg2+) 3. iron-containing preparations
because divalent cations inhibit drugs’ absorption in the gut.
518
whats the PCWP like in ARDS?
normal PCWP!!!!!!!!!!!!!!!!!!!!!!!!
519
whats the characteristic **CD4: CD8 t cell ratio** (noted in bronchoalveolar lavage fluid) seen in a disease mostly affecting **African american** womens whos histological characteristic feature includes: **Schaumann and Asteroid bodies?**
this is **Sarcoidosis** and the ratio is **CD4:CD8 \>2:1!!!!** **so more CD4+T cells!!!!!!!!!!!!!**
520
look at this picture Significantly distended loops of small bowel within the abdomen and pelvis **with curvilinear lucencies** surrounding **several loops of dilated small bowel** in the right side of the abdomen is called what and is pretty much diagnostic for what infantile condition?
**Pneumatosis intestinalis** (also called intestinal pneumatosis) is gas cysts in the bowel wall. As a radiological sign, **In newborns, pneumatosis intestinalis is considered diagnostic for _necrotizing enterocolitis!!!!!!!!!!!!!!!!!!!!!_** This is in contrast to gas in the intestinal lumen (which is relieved by flatulence). **In newborns, pneumatosis intestinalis is considered diagnostic for necrotizing enterocolitis,** and the air is produced by bacteria in the bowel wall.
521
**generalized Diffuse. *_loss of consciousness (unresponsive)..both hemis all ove brain._* _unlike_** _partial/focal ones that only affect ONE part/area of your brain_ **Types:** **ƒ Absence (petit mal)**—**3 Hz, *no postictal*** ***confusion*, blank stare** ƒ **Myoclonic**—quick, repetitive jerks ƒ **Tonic-clonic (grand mal)—**alternating stiffening and movement (jerky) ƒ **Tonic**—stiffening ƒ **Atonic**—“drop” seizures (falls to floor); commonly mistaken for fainting; floppy
522
whats the main AE of the following drug **5-lipoxygenase pathway inhibitor.** Blocks conversion of arachidonic acid to leukotrienes.
hepatoxic **Zileuton**
523
if a patient has an allergic reaction and develops urticaria (hives) WHAT part of the skin is affected and how? epidermis (superficial) dermis deep dermis and subcutaneous tissue
JUST THE (superficial) **DERMIS**!!!!!! igE mediated degranulation of mast cells and basophils--\> INCREASED PERMEABILITY OF MICROVASCULATURE AND **EDEMA OF THE SUPERFICIAL DERMIS!!!!!!!** NOTE!!! if the subcut or deeper dermis **is affected this is ANGIOEDEMA**
524
causes of methhemboglobinemia
Nitrites (eg, from dietary intake or polluted/high altitude H2O) and benzocaine cause poisoning by oxidizing Fe2+ to Fe3+.
525
Self-limited disease often following a u-like illness (eg, viral infection). May be **hyperthyroid** early in course, **followed by hypothyroidism.** Histology: granulomatous in ammation. Findings: ESR, jaw pain, very tender thyroid. DIAGNOSIS?
Subacute granulomatous thyroiditis (de Quervain)
526
why is this the answer?
527
neurotoxin that blocks exocytosis of Ach-containing pre-synaptic vesicles from peripheral nerve terminals causing cranial and peripheral nerve palsies, muscle weakness and respiratory paralysis what bug makes it
Clostridium BOTULIN motile obligate anaroebe
528
when a kid btw the ages of 1-4 is holding onto his caretakers hand and falls down OR tries to run into the street then holds his/her forearm in an EXTENDED and PRONATED position what LIGAMENT injury is commonly assoicated with displaement of what BONE what is this injury called classically?
NURSEMAIDS ELBOW EFFED UP THE RADIAL HEAD VIA SUBLUXATION AND THEN THE ANNULAR LIGAMENT TEARS OFF AND UP TO GET STUCK IN BETWEEN WHERE THE PIC SHOWS \*not common after age 5 bc the ligament gets tougher and more stable
529
DOES VITAMIN E enhance OR suppress the anticoagulant effects of warfarin? hmmmm.
Can ***_enhance_*** anticoagulant effects of warfarin.
530
due to an inactivating mutation in **BMPR2 gene** (normally inhibits vascular smooth muscle proliferation); poor prognosis. what disease?
Heritable PAH (Pulmonary arterial hypertension )
531
The boards LOVES the association between hep C and vasculitis and nephrotic syndrome secondary to cryoglobulinemia. This question goes a step further by asking for the type of hypersensitivity reaction. Cryoglobulinemia can lead to small-vessel vasculitis, which can cause purpura.
532
when is TDT+ postive?
on immature LYMPHOCYTES so PRE-t and PRE-b cells NOT mature lymphocytes and NOT on myeloid cells so its positive in **ALL (b-all & t-all)**
533
what are **two complications** associated with the MSK diseases that are tied to the following ABs ## Footnote **Anti-Jo-1 (whats another name for this antibody)?** **anti-SRP** **anti-Mi-2** **Antinuclear (ANA)**
diagnosis: **Polymyositis, dermatomyositis** **Anti-Jo-1 = aka: Antihistidyl-tRNA synthase** **two complications=** **1. myocarditis** **2. interstitial lung disesae**
534
1. incomplete obliteration of the processus vaginalis in male=? 2. incomplete fusion of the (urethral) urogenital folds in males =? 3. faulty positioning of genital tubercle @ 5 weeks gestation in male =? 4. Malunion of labioscrotal folds in males=?
1. hydrocele 2. Hypospadias 3. Epispadias 4. Bifid scrotum
535
if a pt is taking pyridostig for MG but it gives them Diarrhea, what can you give them to fix is?
anti M's SCOLPAL**AMINE** HYOSCY**AMINE** DICYCLO**MINE** **NOT PILOCARPINE! (thats a M agonist!)** **and its nice bc these M blockers listed above dont interfere with the NMJ where pyridostig is working bc there they have NIcotinic not muscarinic receptors! yay**
536
whats the MOA of Cilostazol ?
**Phosphodiesterase III inhibitor-**-\> **inc cAMP** in platelets, resulting in **inhibition of platelet aggregation** *(PDE in endothelium usuall breaks down cAMP which is needed for vasodilation)* **and** is a **direct arterial vasodilator** **Used for: Intermittent claudication, coronary vasodilation, prevention of stroke or TIAs (combined with aspirin), angina prophylaxis.**
537
a 10 year old child has anemia SINCE birth his spleen is 5x the norma size MHCH is INCreased what is the diagnosis and what would be the appropriate treatment
hereditary spherocytosis **SPLENECTOMY!!!** Spherocytes, no central pallor. (note: splenectomy is NOT indicate thal major and Fetal HbF is protective til 6months so no anemia til then and **target cells** are seen with **anisopoikilocytosis**
538
NEUTROPHILS with BILOBED NUCLEI SEEN AFTER LONG STANDING CHEMOTHERAPY IS CALLED WHAT AND IS ASSOCIATED CLASSICALLY WITH WHAT heme disorder?
**Pseudo–Pelger-Huet anomaly**—neutrophils with bilobed nuclei. Typically seen after chemotherapy. **associated with: Myelodysplastic syndromes**
539
what kind of immune cells use PERFORIN and GRANZYMES to induce apoptosis in infected cells? what two main types of cells are these cells targeting?
NATURAL KILLER CELLS and they target VIRALLY infected cells and tumor cells!
540
the embryological structure within the heart that received blood from the vena cava is the\_\_\_\_\_ in adults (the RIGHT horn) of this strucutre forms the smooth portion of the RA and is knows as the\_\_\_\_\_ well then what does the LEFT horn become?
Sinus venosus Sinus venarum Coronary sinus
541
whats the diff btw the following two following IMMATURE defense mechanisms? Displacement vs projection
**Displacement**: angry recently divorced dad *takes ange*r out on innocent kids vs **Projection**: Husband who is cheating or wants to cheat blames his wife of cheating
542
morbid obesity present as Restrictive lung diseases due to these pts having a Poor structural apparatus. in an obese, how is the **ERV** specifically affected and how does this effect the **FRC?**
**ERV is DECREASED greatly** ## Footnote if we say that **Expiratory reserve volume** is the Air that _can still be breathed out after normal expiration_ _and_ **RV + ERV= FRC** **then a dec in ERV--\> a decrease in FRC bc** **Functional residual capacity** is the Volume of gas in lungs after normal expiration; includes RV, *cannot be measured on spirometry*
543
what cell marker located on macrophages is the receptor for PAMPs? whats an example of a PAMP?
CD14!!!!!!! aka: TLR-4! LPS of gram -ve bacteria! (thymus independent) Endotoxins/LPS DIRECTLY stimulate macrophages by binding to their exotoxin receptor. NO T helper cell involved \*\*\*UNLIKE SUPERANTIGEN MOA\*\*\*
544
out of all the INHALED ANESTHETICS, the following have specific AEs, vat are they? (halothane) , (methoxyflurane) , (enfurane) , (N2O)
Hepatotoxicity **(halothane)** nephrotoxicity **(methoxy urane)** proconvulsant (**enflurane),** expansion of trapped gas in a body cavity **(N2O)**.
545
**3 A’s**: **neuromuscular Activity** (clonus, hyperreflexia, hypertonia, tremor, seizure), **Autonomic stimulation** (hyperthermia, diaphoresis, diarrhea), and **Agitation.** **describes NMS or Serotonin synd?** **whats the main diff? and whats the antidote for each?**
**Serotonin syndrome was described** **antidote= cyproheptadine (5-HT2 receptor antagonist).** Neuroleptic malignant syndrome (NMS)— **RIGIDITY!!!!!, myoglobinuria,** autonomic instability, hyperpyrexia. antidote= **dantrolene**, D2 agonists (eg, **bromocriptine)**.
546
* Enlargement of **existing** *ACTH-secreting _pituitary adenoma_* **after** bilateral adrenalectomy **for refractory Cushing disease** (due to removal of cortisol feedback mechanism) * Presents with **hyperpigmentation,** **headaches** and **bitemporal hemianopia.** * Treatment: pituitary irradiation or surgical resection. ## Footnote **what disease is this? think of a black philanthropist**
**Nelson syndrome**
547
whats the diff btw hypertensive URGENCY and EMERGENCY
**urgency** is **NO ORGAN DAMAGE** sxs with a **BP \>180-120** **Emergency** severe **HTN (eg: 225/115)** **with END ORGAN DAMAGE** (eg: pappiledema, pulmonary edema, aortic dissection, AKI (elevated Cr--Fenoldopam real food for this), encephalopathy (no focal sxs but confusion), Acute coronary syndrome
548
which bug has the following characteristics: ## Footnote **Cord factor** creates a “serpentine cord” appearance in virulent strains; inhibits macrophage maturation and **induces release of TNF-α.** **Sulfatides** (surface glycolipids) **inhibit phagolysosomal fusion.**
(VIRULENT) **MYCOBACTERIUM TB** not found in avirulent strains Cord factor presence increases the production of the cytokines interleukin-12 (IL-12), interleukin-1 beta (IL-1β), interleukin-6 (IL-6), tumor necrosis factor (TNFα), which are all pro-inflammatory cytokines important for granuloma formation. IL-12 is particularly important in the defense against M. tuberculosis; without it, M. tuberculosis spreads unhampered.[30][31] IL-12 triggers production of more cytokines through T cells and natural killer (NK) cells, while also leading to mature Th1 cells, and thus leading to immunity.[32] Then, with IL-12 available, Th1 cells and NK cells produce interferon gamma (IFN-γ) molecules and subsequently release them.[33] The IFN-γ molecules in turn activate macrophages.[34] When macrophages are activated by cord factor, they can arrange into granulomas around M. tuberculosiscells.[13][35] Activated macrophages and neutrophils also cause an increase in vascular endothelial growth factor (VEGF), which is important for angiogenesis, a step in granuloma formation
549
Susceptibility is often inherited as **autosomal dominan**t with variable penetrance. **Mutations in voltage-sensitive ryanodine receptor cause Ca2+** release from sarcoplasmic reticulum. what does this cause? what can you give to help?
**Malignant hyperthermia**—rare, life-threatening condition in which inhaled anesthetics or succinylcholine induce fever and severe muscle contractions. Treatment: **dantrolene,** a **ryanodine receptor antagonist.**
550
list the 3 loop diurectics and their main AE's ## Footnote **OH DANG!**
**Furosemide** **bumetanide** **torsemide** **Ototoxicity,** **Hypokalemia,** **Dehydration,** **Allergy (sulfa)/metabolic Alkalosis,** **Nephritis (*interstitial)*,** **Gout.**
551
**ATP7B gene**; chromosome **13** = what diseae **C282Y** chromosome **6** = what diseae
ATP7B gene; chromosome 13 = **Wilson disease (hepatolenticular degeneration)** C282Y chromosome 6 = **Hemochromatosis**
552
which **water soluble vitamin** is a vital component of a cofactor used for the following enzyme reactions?ƒ **Pyruvate dehydrogenase** (links glycolysis toTCA cycle) **ƒ *_α-ketoglutarate dehydrogenase_*** *_(TCA cycle)-especially known for this_* ƒ **Transketolase** (HMP shunt) **ƒ Branched-chain ketoacid dehydrogenase**
**Vitamin B1 (thiamine) used in the cofactor _Thiamine pyrophosphate (TPP)._**
553
AE for common antihypertensives
554
what is: Catharanthus roseus, commonly known as the **Madagascar periwinkle**, rosy periwinkle or teresita and whats the danger in using this natural supplement?
The substances vinblastine and vincristine extracted from the plant are used in the treatment of leukemia [16] and Hodgkin's lymphoma. so a person on chemo who has BM suppressio should not take it!
555
alpha thal creates abnormal tetramers of beta (HbH) and gamma tetramers (Barts) which are most similar to HbF Myoglobin HbC
MYOGLOBIN!!! these have SUPER high affinity for O2 (UNLIKE FETAL Hb) and therefore, cannot unload O2. this is very bad
556
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557
List 5 main causes of **Pulmonary HTN** and list specific diseases in these categories too
**Pulmonary arterial hypertension** [idiopathic or heritable-inactivating mutation in BMPR2 gene] **Left heart disease** [systolic/diastolic dysfunction and valvular disease (eg, mitral lung).] **Lung diseases or hypoxia** [Destruction of lung parenchyma (eg, COPD), hypoxemic vasoconstriction (eg, obstructive sleep apnea, living in high altitude).] **Chronic thromboembolic** [Recurrent microthrombi--\> cross-sectional area of pulmonary vascular bed] **Multifactorial** [Causes include hematologic, systemic, and metabolic disorders.]
558
NEGATIVE LABS (normal ESR, CR, etc) widespread GENERALIZED PAIN disproportionate to clinical findings poor stage 4 sleep FATIGUE!!!!!! main sx Most commonly seen in females 20–50 years old. cognitive disturbance
Fibromyalgia!!!
559
how do proteins know if they are destined to be translated in the RER vs somewhere else in the cell?
they possess a HYDROPHOBIC N TERMINAL PEPTIDE SEQUENCE THAT IDENTIFIES THEM AS SUCH---if these sequences are removed, protein will accumulate in the CYTOSOL
560
what are three main uses of **MISOPROSTOL**
A **PGE1 analog**. INC production and secretion of gastric mucous barrier, DEC acid production. Prevention of NSAID-induced peptic ulcers (NSAIDs block PGE1 production); maintenance of a PDA. Also used off-label for induction of labor (ripens cervix).
561
**cytokeratin** and ***_CALRETININ +ve_* cancer** **theres only one!**
**MESOTHELIOMA!!!!!** **fun fact: smoking is NOT A RISK FACTOR FOR IT!!!!!** **but asbestos is!!!!!!!!!!!!!!!!!!!!!**
562
Match the following histopatholocal changes with its respective disease: - **Macrovesicular fatty change** - **Swollen and necrotic hepatocytes**S with neutrophilic infiltration & fibrosis. Mallory bodies B (intracytoplasmic eosinophilic inclusions of damaged keratin laments). - **Micronodular, irregularly shrunken** liver with “hobnail” appearance. **Sclerosis around central vein (zone III).** - **Thrombosis or compression of hepatic veins with centrilobular congestion and necrosis** - **Panlobular microvesicular steatosis (lacking necrosis or inflammation)** - **Fatty infltration of hepatocytes** A cellular **“ballooning” and eventual necrosis.** **a. Alcoholic cirrhosis** **b. Non-alcoholic fatty liver disease** **c. Hepatic steatosis** **d. Alcoholic hepatitis** **e. Reye Syndrome** **f. Budd Chiari**
Macrovesicular fatty change-**_Hepatic steatosis_** Swollen and necrotic hepatocytes with neutrophilic in ltration. **Mallory bodies** (intracytoplasmic eosinophilic inclusions of damaged keratin laments).-**_Alcoholic hepatitis_** Micronodular, irregularly shrunken liver with “hobnail” appearance. Sclerosis around central vein (zone III).-**_Alcoholic cirrhosis_** Thrombosis or compression of hepatic veins with centrilobular congestion and necrosis-**Budd Chiari** Panlobular ***_microvesicular_*** steatosis (no necrosis or inflammation!); on EM, the number of mitonchondria are reduced too-**Reye Syndrome** Fatty in ltration of hepatocytes A cellular “ballooning” and eventual necrosis-**Non-alcoholic fatty liver disease**
563
**Decreased URINARY ca2+** in the face of **Hyper**calcemia with High to **normal levels of PTH**...what is the first diagnosis that should come to mind?
Familial **hypocalciuric hypercalcemia** * **Defective Ca2+-sensing receptor** (CaSR) in multiple tissues (eg, **parathyroids, kidneys**). * Higher than normal Ca2+ levels *required* to suppress PTH. * Excessive renal Ca2+ reuptake mild hypercalcemia and **hypo**calci*uria* with *normal to PTH levels.* * do not confuse with pseudohypoparathyroidism* * which is called pseudo bc PTH is actually High (this is a type of seconary hyperparathyroidism)...but the body is acting as thougth there is low pth which is why it has this name.* * importanttly (the type 1) thats called albright hereditary osteodystrophy (is only gotten from MATERNAL imprinting)...this gene mutation in the mom affects the kidney so her kids will have end organ PTH resistnace and present the HYpocal, (due to the Hyperphos) and inc PTH (since body thinks it has low PTH) + phenotypic features* * BUT the dad will only pass on the Phenotypic exam exams features like shorted 4th/5th digits, intell disabilyt, short stature BUT his kids kidneys are ok so lab values are fine*
564
what carcinogen can lead to squam cell carcinoma
arsenic!
565
**MELANOMA** often driven by activating mutation, in **BRAF kinase (serine thronine kinase)=an oncogene.** Metastatic or unresectable melanoma in patients with **BRAF V600E mutation** may benefit from\_\_\_\_\_\_\_\_\_\_**,** a **BRAF kinase inhibitor.**
**VEmuRAFenib** Small molecule inhibitor of **BRAF oncogene ⊕ melanoma.** **VE**mu**RA**F-enib is for **V**600E- mutated B**RAF** inhibition.
566
what are foveolar cells
**Foveolar cells or surface mucous cells** are mucus-producing cells which cover the inside of the stomach, protecting it from the corrosive nature of gastric acid.These cells line the gastric mucosa (mucous neck cells are found in the necks of the gastric pits). The mucus-secreting cells of the stomach can be distinguished histologically from the intestinal goblet cells, another type of mucus-secreting cell.
567
whats a complication of: Secondary biliary cirrhosis and what is it even? and what are a couple RFs?
**Extrahepatic** biliary obstruction (gallstones, biliary strictures, pancreatic carcinoma). )--\> INC pressure in **intrahepatic ducts injury/ fibrosis and bile stasis** May be complicated by **ascending cholangitis.**
568
what **two electrolyte disturbances** are likely to be seen in a pt taking amphotericin B (due to its renal tox) what renal tubular defect is it mostly associated with? MOA: binds to ergosterol in cell membranes and pokes holes into it
**HYPO** Mg2+ **HYPO** K+ reflects increased DIstal tubule permeability ***_Distal renal tubular acidosis (type 1)_*** Defect in ability of **α intercalated cells to secrete H+** **--- inc PH of urine (unique feature) + hypokalemia**
569
homozygous mutation of the gene for WHICH enzyme is the most common genetic cause of elevated homocystein levels in serum (Hyperhomocysteinemia) - what cofactor does this enzyme use - it helps in the conversion of homocysteine to \_\_\_\_\_\_\_\_
**Methylene Tetrahydrofolate Reductase (MTHFR)** **-uses FAD as a cofactor** **-homocystein----remethylated----\> *Methionine*** *note: [remethylation occurs with the donation of a methyl group from MTHFR via Methionine Synthase) with vit b12 (cobalmin as the cofactor)*
570
are **pamps** on apc's or part of the offending agent/invader/antigen? are **TLCs** (toll like receptors) on apc's or on offending cell? what are **3 specific examples** of PAMPs?
**Toll-like receptors (TLRs)**: are pattern recognition receptors **ON APCs** (mac, dendridic cells, etc) that recognize pathogen-associated molecular patterns **(PAMPs)** on **Ag/invader/offending agent**! Examples of PAMPs include 1. **LPS** in (gram ⊝ bacteria) 2. **Flagellin** in (bacteria) 3. **N****ucleic Acids** in (viruses).
571
a patient with **MEDIASTINAL WIDENING SEEN ON XRay (like the aortic park is real wide)** with **SUDDEN** onset **severe** pain and **REAL HIGH BP (bc non compliant with meds for eg)---eg: 180/120 (LOOK AT THAT DIASTOLIC NUMBER!)** **NORMAL EKG** what do you HAVE TO THINK?
**AORTIC DISSECTION!!!!!!!!!!** due to long standing UNCONTROLLED HTN seen in MARFANS too bicuspid aoritc valve (inherited)
572
whats the diff btw these two pink things? where are they seen?
**Parkinsons** **Lewy bodies** (composed of **α-synuclein**—intracellular **eosinophilic inclusions** **Alzheimer disease** Neuro brillary tangles/ intracellular**, hyperphosphorylated tau protein** = i*nsoluble cytoskeletal elements*; number of tangles correlates with degree of dementia. (look like tears)
573
what is it when a child has excess growth hormone (somatotropin)? whats the most common cause of death in children with inc GH?
**gigantism** [vs. acromegaly in adults] **most common COD= HF!!!**
574
if a pt is on **chemo**, has **HIV**, or **anorexia** and their appetite needs to be stimulated or also if soomeone is on chemo and they get nausea but mostly think of this substance STIMULATING APPETITE...**.what is this substance that should be given?**
**Marijuana (cannabinoid)** _***\*Dronabinol\****_ Pharmaceutical form is dronabinol (tetrahydrocannabinol isomer)=**therapeutic THC** **used as antiemetic (chemotherapy)** **appetite stimulant** (in ***AIDS)** & **chemo & anorexia***
575
Autosomal recessive. * Deficiency of large neutral amino acid (eg, tryptophan) transporters in **proximal renal tubular** cells and on **enterocytes** * leads to **neutral aminoaciduria** and DECREASED absorption from the gut * leads to **DECREASED tryptophan** for conversion to **niacin** * leads to **pellagra-like symptoms.** * Treat with high- protein diet and nicotinic acid. what disease is this? what does this characteristically present like?
Hartnup disease presents like: * Diarrhea, * Dementia (also hallucinations) * Dermatitis (C3/C4 dermatome circumferential “broad collar” rash [Casal necklace], hyperpigmentation of sun- exposed limbs
576
if a pt is getting **IV NE** bc they are hypotensive and have lactic acidosis and then they develop a 1. blanching vein surrouding the IV site 2. surround tissues become cold, hard, pale what is this? and HOW DO YOU TREAT IT?
this is **NE extravasation** (causes a1 receptor mediated vasoconstriction & tissue necrosis) **TREAT: PHENTOLAMINE** an a1 blocker B2 + like Isoproterenol can vasodilate also BUT there are alot more a1 receptors SUBCUTANEOUSLY. vs. B2 are more in striated muscle, renal, and mesenteric vasc beds so they dec PVR and inc CO
577
signs of embolism (Livedo reticularis) following an invasive procedure (angioplasty, angiography) in an elder person with a history of atherosclerosis is suspicious of **what complication (the most common especially) in what locations** **and would importantly look like what on histology?**
first off: **Livedo reticularis** is a common skin finding consisting of a **mottled** reticulated vascular pattern that appears as a **lace-like purplish discoloration** of the skin. The discoloration is caused by swelling of the venules owing to obstruction of capillaries by small blood **clots.** this is suspect of **ATHERO-EMBOLIC DISEASE** (THINK ABOUT THE NAME) -during that invasive procedure like aortic catheritizatoin, **CHOLESTEROL containing debris can DISLODGE** and **travel to smaller vessels where they block the area and cause ischemia to that tissue** which presents a few days to weeks post surgery as: ***_MOST COMMONLY:_*** acute kidney injury!!!! (oliguria and azotemia)----THE PATIENT **WOULD NOT** HAVE A FRANK INFARCTION WITH HEMATURIA AND FLANK PAIN AND THE WHOLE SHABANG BC CHOLESTEROL **EMBOLI ARE WITTLE.** ON HISTO (if in the kidney) you could see **needle shaped cholesterol clefts** that **partially or completely obstruct the arcuate or intralobular renal arteries** other organs that can be involved are: GI, CNS (like retina vessels)
578
diagnosis? bilateral shrunken kidneys use nsaid for 8 years for knee pain microscopic hematuria sterile pyuria bun: 12 cr: 6
**CHRONIC INTERSTITIAL NEPHRITIS!** "analgesic nephropathy" nsaid damage tubules directily after a while through uncoupling oxid phos and by decreased PG synth causing constriction of medullary vasa recta and subs pap necrosis i got tricked on this bc i didnt see eosinophila and thought of "acute interstitial nephritis" also note that if you look at BUN: C its less than 15 telling you an INTRArenal issue (like a TUBULAR issue) bc cant absorb proper amt of urea (ration is supposed to be 15)
579
**Essential fatty acids...name 2**
2. **Essential fatty acids** b. Functions of **linolenic acid (ω-3)** and **linoleic acid (ω-6)** **(1) Important in the synthesis of eicosanoids (** **• Eicosanoids are important in muscle contraction/relaxation, blood vessel constriction/relaxation, blood clot formation, blood lipid regulation, and immune response to injury and infection, including fever, in ammation, and pain.**
580
whats the name of the drug that fits the following description: Expectorant—thins respiratory secretions; does not suppress cough re ex.
Guaifenesin
581
how do the levels of estrogen and FSH CLASSICALLY change in MENOPAUSE?
decreased estrogen **INCREASE in FSH**!!!! is DIAGNOSTIC!!!!!!!
582
Mutations at different loci can produce a similar phenotype is what genetic principle? give 2 clincal examples
**Locus heterogeneity** ## Footnote **1.** **Albinism.** **2.** **Osteogenesis Imperfecta** (eg: 2 genes on chr, 17 and 1 gene on chr. 7---\> both lead to OI type 2 that are indistinguishinable from one another despite coming from idff loci. same phenotype, diff loci. **3. Ehlers Danlos** **4. Tuberous Sclerosis** **5. Retinitis Pigmentosa**
583
whats the test of choice for PULMONARY EMBOLI?
CT PULMONARY ANGIOGRAPHY LOOKING FOR FILLING DEFECTS
584
a man who has a history of COPD and is on chronic prednisone AKA: is immunocompromised has pseudomonas pnm, what could you give to him? tell me 3 options
585
does wallerian degeneration (axonal degeneration and breakdown of myelin sheath distal to the injury with subsequent clearance of myelin and an opporuity for REGENERATION)) take place in the CNS (oligos) or just the PNS (shwanns)?
just the pns bc in the CNS the buildup and persistnace of myelin debris (mac cant cross BBB so quicly or easily), secretion of inhibitory neuronal factors and dev of dense glial scar both by astrocytes DONT allow regeneration!
586
cardiac arrhythmias (bradycardia and junctional escape beats d/t AV nodal block) HYPERKALEMIA **COLOR VISION ALTERATIONS** what drug toxicity?
DIGOXIN
587
how are levels of inhibitory neurotransmitters and excitatory NTs altered in hepatic encephalopathy? very distinct pattern. what are 6 major preceipitating factors in hep encep? whats a really big one thats targeted by meds?
GABA (inhibitory) is **INCREASED** (too much!) Glutamate (excitatory) is **DECREASED** (not enough!) know that ***_GI bleeding (alchoholic comes in with GI bleed; eg: vomiting bright red blood)_*** is a huge one that **INCREASED the LOAD OF NITROGENOUS** substances (in the form of Hb that is then convereted to ammonia) and absorbed by the gut and thats bad!!!! know the other RFs too!
588
in acromegaly, are the serum levels of IGF-1 high or low how would you use glucose to diag Acrogmegaly?
HIGH! IGF-1 is made by liver and promotes growth Glucose is SUPPOSED to SUPPRESS GH (somatotropin) so if you give Glucose and GH doesnt decreaes then ACROMEGALY
589
Migratory (superfiicial) Thrombophelbitis, known as Trousseau Syndrome, should raise suspicion for what type of disease process?
CANCER
590
## Footnote Recombinant uricase enzyme that catalyzes metabolism of uric acid to **allantoin** (a more water-soluble product). **Inhibits reabsorption of uric acid in proximal convoluted tubule** (also inhibits secretion of penicillin). AE: *Can precipitate uric acid calculi.* name these each
**Pegloticase:** metabolism of uric acid to **allantoin** (a more water-soluble product). **Probenecid**: **Inhibits reabsorption of uric acid in proximal convoluted tubule** (also inhibits secretion of penicillin). AE: *Can precipitate uric acid calculi.*
591
DIAGNOSIS? ## Footnote Early **changes in personality and behavior** (inc impulsivity is the "Behavioral variant", **or aphasia** (decreased fluency but memory in tact) is seen in the: "primary progressive aphasia" type May have associated movement disorders (eg, parkinsonism, ALS-like UMN/LMN degeneration). **Previously known as Pick disease.**
**Frontotemporal dementia** ## Footnote **SEEN IN HISTO:** severe atrophy, neuronal loss, gliosis (severely atrophic, sometimes paper-thin gyri in the temporal and frontal lobes) and presence of abnormal neurons (Pick cells) containing inclusions (Pick bodies= Inclusions of hyperphosphorylated tau (round Pick bodies ) FTD **affects personality, behavior, and usually language function (syntax and fluency) while memory is in tact and impaired in** *Alzheimer's disease rather*
592
Decreased serum iron and ferritin and increased serum transferrin levels confirm the diagnosis of...
**iron def anemia**
593
CD10+ associated with Down syndrome after age 5
**ALL!!!!!!!!!!!!!! B-ALL specifically!!!** bc CD10+ve!!!! Peripheral blood and bone marrow have SUPER INCREASED lymphoblasts **TdT+** (marker of pre-T and pre-B cells), **CD10+** (marker of pre-B cells).
594
what two types of kidney stones need INC ph?
**1. Calcium phosphate** **2. ammonium mg phosphate-**--**STRUVITE-**--(from urease +ve bugs) **3. Ca2+ oxalate too** bc a DEC ph lead to less citrate in pee and WE NEED CITRATE to buffer Ca2+ in pee. so this needs an **Inc PH as well.** note **PHOSPHATE is involved and ca2+ as well**
595
widened pulse pressure leading to head bobbing + head pounding with exertion what murmur
**AORTIC REGURG**
596
Selenium is an antioxidant used in what biochem rxn?
• Component of **glutathione peroxidase**, which produces reduced glutathione, an antioxidant that converts hydrogen peroxide to water
597
what **muscle** is the main player when a pt is doing a **VALSALVA** to, for example, help with PAROXYSMAL SVT? if the Paroxsymal SVT is not helped with that what med can you use?
**RECTUS abdominus** is the main muscle used in **VALSALVA** can give **ADENOSINE (dec the rate of diastolic depol and decreased HR)**
598
how do tell/what are the diff btw Actinic keratoses & seborrheic keratoses?
Actinic keratoses usually **pink or red, poorly marginated, and scaly** on palpation, although some are **light gray or pigmented, giving them a brown appearance.** They **should be differentiated** from seborrheic keratoses which increase in number and size with aging. Seborrheic keratoses tend to appear **waxy and stuck-on** but can often take on an appearance similar to actinic keratoses. Unlike actinic keratoses, ***_seborrheic keratoses also occur on non-sun-exposed areas of the body and are not premalignant. =very different from_*** **Actinic keratoses !!!!!!!!!!**
599
point mutation in a **SPLICE SITE** commonly results in what diseae? **Tay sachs** (CHERRY RED SPOT ON MACULA-exosaminidase A deficiency) and **DUCHENNE** (but not beckers bc its milder) are a result of what type of mutation in DNA?
**B-THALASSEMIA** (also can be due to a mutation in the promoter region) **FRAMESHIFT MUTATIONS!**
600
Pain and stiffness in shoulders and hips, often with fever, malaise, weight loss. **Does not cause muscular weakness.** More common in women \> 50 years old; **associated with giant cell (temporal) arteritis!!!!!!!!!!!!!!!!!!!!!!!!!!** **INC ESR, INC CRP, *normal CK.***
**Polymyalgia rheumatica**
601
what viral infection is knows to cause cholecystitis?
cmv
602
stereotyped hand wringing **x linked dom** almost exclusively affects girls onset age 1-4
Rett syndrome
603
**chocolate-colored blood.** **what should you think of immediately**
**Methemoglobinemia**
604
aside from systemic fungal infections, absolute neutropenia (febrile neutropenia) in setting of infection can be seen with what type of bug?
freaking gram -ves! especially **PSEUDOMONAS!!!!!** it gives you **ecthyma gangrenosum!!!!** (which looks like DIC kinda but lab values dont match!) you will be give leukocute count of like 800 \*real low\* and then told its 60% neutrophils so neutrophils are 480 total and thats less than 500 and thats real low burn pts, NEUTROPENICS, indwelling catheterrs. hospitalized are very susceptible
605
Associations! name that bug Latin america systemic mycoses dimorphic "captains wheel formation" \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ opportunistic fungi commonly causes meningoencephalitis via hematogenous spread NOT dimorphic \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Desert Rheumatism=arthralgias Desert bumps=Erythema nodosum mold in the cold, heat in the yeast (dimorphic)
**Paracoccidioidomycosis** **Cryptococcus** **Coccidioidomycosis**
606
12 year old presents with high blood sugar at fasting and post prandial has a long fam history of diabetes but they have all been able to maintain a good level of glycemic control with JUST dietary modifications what enzyme is most deficienct in the patient?
**GLUCOKINASE!!!!!!!!** pts has **MODY** (maturity onset diab of the young)-**inherited** \*\*\*\*this is **easily controlled with dietary mids!** basically glucokinase is **ONLY in liver and PANCREAS** and has low aff low glucose. so when glucose is high, glucose (**via GLUT 2)** transporter it (glucokinase) catalyzes the first step in GLYCOLYSIS--\> kreb--\> ATP--\> causes K channel to close so no more K comes into cell (causes depol) --\> Ca2+ comes into cell---\> **exoctyosis of INSULIN.** **so if not glucokinase, this does not occur in the panc beta cells--\> hyperglycemia** note: in the liver, **glucokinase** works to help activae glycogen synthesis (so it has diff roles in panc and liver).
607
PGF2a drug that INC OUTFLOW of aqueous humor and this is a treatment for GLAUCOMA
**(PGF2a )** **Latanoprost** **Bimatoprost,** **AE: Darkens color of iris (browning), eyelash growth**
608
What are the 2 main GI related COMPLICATIONS IN ACROMEGALY?
INC risk of 1. **colorectal polyps** 2. **GI cancer**
609
how does having **Sjögren syndrome affect digestion of carbs**
Enzymatic digestion of carbohydrates (CHOs) a. **Digestion begins in the mouth.** (1) **Amylase** from the salivary glands breaks down **polysaccharides** (e.g., starch) into **disaccharides** (lactose, maltose, sucrose). (2) In **Sjögren syndrome**, the salivary glands are destroyed and **amylase is not available to digest polysaccharides.**
610
which of the following types of muscle cells LACK troponin? As a result, what messenger protein which binds Ca2+ is used by this type of muscle during excitation contraction coupling? Cardiac muslce Smooth muscle Skeletoal muscle
Smooth muscle Calmodulin
611
in an HIV positive ind, how can you tell btw **toxo** **EBV** **JC reactivation (PML)** on imaging?
**Toxo**= **MULTIPLE** *ring-enhancing l*esions on MRI **EBV= CNS lymphoma**—*ring enhancing****,* may be SOLITARY** *_vs Toxoplasma)_* JC reactivation= **(PML)= NON ENHANCING areas of *demyelination* on MRI** on imaging?
612
what primary immune deficiency is associated with increased **AFP levels?**???? whats the CLINICAL TRIAD ASSOCIATED with this disease?
Ataxia-telangiectasia Defects in ATM gene failure to repair DNA double strand breaks cell cycle arrest. Triad: 1. cerebellar defects-PURKINJE cells layer--\> (Ataxia), 2. spider Angiomas in the EYE (telangiectasia), 3. IgA defciency (DEcrease in Ig, A, G E)
613
on pap smear, a cluster of basophilic thin filaments that reemble **COTTON CANDY** can be an incidental finding in pts with\_\_\_\_\_ what organism is this?
on pap smear, a cluster of basophilic thin filaments that reemble COTTON CANDY can be an incidental finding in pts with\_**\_IUDs**\_\_\_ **actinomyces like organisms**
614
which two hep viruses are naked and what does this mean? are they destroyed or not destroyed in the gut?
A & E are non env and hence are NOT destroyed in the gut
615
what would you be testing for if you did a test where you take amniotic fluid+ethanol and shake it to see that a stable ring of foam appears? what substance would cause this observed finding?
you are testing for lung maturity and presence of SURFACTANT causes the ring of foam \*additionally you can do - surfactant to albumin ratio - slide agglutination for phosphatidylglycerol - and the common lecithin: spingomyelin ratio
616
**what glaucome drug works by DECREASING AQEUOUS HUMOR VIA WAY OF VASOCONTRICTION** ***_BUT IS CONTRAINDICATED IN CLOSE angle glaucome!_***
**Epinephrine!!!!!!!!!**
617
which is the only bacterial DNA pol that has 5'--\>3' exonuclease activity so it can remove RNA primers DNA Pol I or DNA pol III
DNA pol I !!!!!!!!!! dna pol III only has 3'---\>5' exonuclease activity and cannot remove RNA primers so it needs DNA pol I in its life.
618
619
descemets membrane where copper builds up in WIlsons, is it in the iris cornea sclera
CORNEA!!!!!!!!!!!
620
what are the 2 two distinct pathways by which transitional cell CA can arise?
Arises via two distinct pathways **Flat-**develops as a **high-grade flat tumo**r and then **invades;** associated with **early p53 mutations (damn aggresive)** **Papillary-**develops as a **low-grade** papillary tumor that progresses to a **high- grade** papillary tumor and **then invades**; *NOT associated with early p53 mutations*
621
name the 3 main txs of PULM HTN and why they work
* **BosENtan:** Competitively **antagonizes ENdothelin-1** receptors--\> DEC. pulmonary vascular resistance. * **Sildenafil:** **Inhibits cGMP PDE-5** and **prolongs vasodilatory effect of nitric oxide.** * **Epoprostenol, iloprost: PGI2 (prostacyclin)** with **direct vasodilatory** effects on pulmonary and systemic arterial vascular beds. **Inhibits platelet aggregation.** **they work bc pulm vasoconstriction thought to be due in part to** **1.** enhanced activity of **thromboxane and endothelin-1 (both vasoconstrictors)** **and** **2.** reduced activity **of prostacyclin and nitric oxide (both vasodilators).**
622
whats the diff btw how respiration is controlled in a normal healthy individual vs someone with COPD as far as the chemoreceptors are concerned? by what mechanism is supplemental O2 working to help a person with COPD?
since pts with COPD have a dec sensitivity to or a blunted response to pCO2 (unlike normal individuals whose central chemoreceptors response to even small increases in pCO2), the pts with COPD respond to O2 so its their PERIPHERAL chemo receptors that drive their respiratory changes. not PCo2 like healthy people. the q had asked what was the cause of the pts respiratory rate DECreasing after receiving supplemental o2
623
Rapidly progressive (weeks to months) dementia with myoclonus (“startle myoclonus”).
**Creutzfeldt-Jakob disease** ## Footnote Spongiform cortex. Prions (PrPc PrPsc sheet [β-pleated sheet **resistant to proteases**]).
624
gray baby syndrom is caused by what antibiotic? whats its MOA? why are infants susucetible to this?
Blocks peptidyltransferase at 50S ribosomal subunit. Bacteriostatic. (BLOCKS FORAMTION OF A **PEPTIDE BOND)** -used in developing nations bc too high risk of aplastic anemia, etc (but its also cheap so good for dev. nations) **USED TO TREAT: Meningitis (Haemophilus in uenzae, Neisseria meningitidis, Streptococcus pneumoniae) and Rocky Mountain spotted fever (Rickettsia rickettsii).** **Ae: Anemia (dose dependent), aplastic anemia (dose independent), gray baby syndrome (in premature infants because they lack liver UDP-glucuronyl transferase).**
625
do vanco, dapto and bacitracin affect cell wall CROSS LINKING AT ALL?
NOOO!!! THEY GENERALLY AFFECT CELL WALL SYNTHESIS DAPTO creates a transmembrane channel and inserts its lipid tail into the membrane and causes depol vanco actually effs up the wall with blocking d ala d ala bacitracin is only topical and prevents phosphorylaiton adn mucopolypeptide transfer into cell wall thus disrpition CELL WALL synth NOTTTT crosslinking (beta lactams do that)
626
what are complement levels like in SLE? WHAT MUCOSAL MANIFESTATIONS DO THEY HAVE
**LOW!!!** **low c3 and c4** **THEY HAVE ORAL OR NASAL ULCERS** antibodies agains self antigens lead to immune complexes forming and depositing in tissues and causing coplement activation
627
whats another way to describe osteoblastic and osteolytic lesions?
osteoblastic aka: SCLEROTIC: due to osteoBLAST stim= more indolent course seen in prostate cancer (african american men at highest risk) osteolytic: LUCENT: due to osteoCLAST stim= more aggressive
628
what are the **3 main ways** you can develop sxs of **PELLAGRA** which you get due to **NIACIN (vit b3) deficiency.** gotta know the pathway of **Niacin synthesis** from **Tryptophan** (even the cofactors)
Just a mild deficiency leads to **GLOSSITIS** Severe deficiency leads to **PELLAGRA** which can be caused by 1. **Hartnup disease** 2. **Malignant carcinoid syndrome** ( tryptophan metabolism to make serotonin depletes tryptophan so then you cant make Niacin 3. **Isoniazid** (vitamin B6). * Symptoms of pellagra: **Diarrhea, Dementia** (also hallucinations), **Dermatitis** (C3/C4 dermatome circumferential “broad collar” rash [Casal necklace], hyperpigmentation of sun- exposed limbs
629
WHATS THE DOC FOR WILSONS DISEASE OR JUST COPPER ACCUMULATION IN GENERAL? MOA? PICK ONE. 1. penicillamine 2. dimercaprol 3. EDTA 4. deferoxamine
**PENICILLAMINE!!!!!!!!!!** dont choose De**fe**roxamine! look at that **FE** in the middle of the name and think of **Fe2+ IRON which is what its an antidote for!!!!**
630
what labs should be drawn before administering STATINS to a pt with hypercholesterolmia? creatinine liver transaminases cortisol levels LPL activity levels
NOT CREATININE!!!!!! (even if you were thinking it bc of the myopathy, if you check creatinine you are looking at RENAL fx and statins are NOT associated with renal AE!!) BUT it IS associated with **HEPATOXICITY SO YOU NEED TO CHECK LIVER TRANSAMINASES!!!!!!**
631
what effect do HIGH dose salicylates and Probencid share in common as it relates to URIC ACID what effect do LOW dose salicylates and DIURETICS (loop diuretics) share in common as it relates to URIC ACID?
what effect do **HIGH dose salicylates** and **Probencid** share in common as it relates to URIC ACID--\> Uric acid **stop** tubular **reabsorption** and **increase EXCRETION** what effect do **LOW dose salicylates** and **DIURETICS** (loop diuretics) share in common as it relates to URIC ACID?--\> **block urinary excretion of Uric acid = bad** **^^^this is why Aspirin is CI in GOUT**
632
**cyclothymic disorder** needs to last for how long to be diagnosed what is it even?
needs to last at least **2 whole years!** its a milder form of biopolar that fluctuates btw mild depression and hypomanic symptoms
633
what drug used for **UC and CD** (the colitis) part is **ACTIVATED BY COLON BACTERIA?**
Activated by colonic bacteria. **Sulfasalazine** A combination of **sulfapyridine** (antibacterial) and **5-aminosalicylic acid** (anti-in ammatory). !!!!
634
Left atrium must push against **stiff LV** wall will cause **what kind of heart sound**? whats a few concrete example of a condition that would cause such a sound?
**s4!!!!!!!!!!!!!!!!!!!**! in **late diastole** (“**atrial kick”**). Best heard at apex with patient in left lateral decubitus position. **High atrial pressure**. **Associated with ventricular noncompliance (eg, hypertrophy)**. \*\*\*Left atrium must push against stiff LV wall. Consider abnormal, regardless of patient age. **heard in: Hypertrophic cardiomyopathy, Concentric Ventricular HTN (eg: from Aortic Stenosis), HTN, Mitral stenosis, Restrictive CM**
635
**Non**sulfonamide Similar to furosemide, but **more ototoxic.**
**Ethacrynic acid**
636
Early lesion is mitral valve \_\_\_\_\_\_\_\_; late lesion is mitral \_\_\_\_\_\_\_\_\_\_\_.
Early lesion is mitral valve **regurgitation;** late lesion is mitral **stenosis.**
637
which viruses are considred INFECTIVE and what makes them this way
## Footnote **Purified nucleic acids of most dsDNA (except poxviruses and HBV) and** **⊕ strand ssRNA (which basically ≈ mRNA) viruses *_are infectious._*** **Naked nucleic acids of** **⊝ strand ssRNA and dsRNA viruses are *_not infectious_*.** **They require polymerases contained in the complete virion. eg: RNA dep RNA pol** **\*NOTE RETRO has a RNA dep DNA pol (reverse transcriptase)**
638
pathogenesis of **YERSINIA PESTIS!** what animals, how does it spread, what does it cause
**BIPOLAR staining; safetly pin, zoonotic, grame -VE coccobaciili. fac intracellular, F1 capsular antigen!!!!** Three mammals fall prey to Yersinia pestis: **wild ro- dents**, domestic city **rodents**, and **human**s. The bacteria reside in the **wild rodent p**opulation between epidemics and are carried from rodent to rodent by the flea. When wild rodents come into contact with domestic city rats (during droughts when wild rodents forage for food), **fleas** can then carry the bacteria to domestic rats. As the domestic rat population dies, the fleas become hungry and search out humans. **During interepidemic periods (we are in one now),** bubonic plague **may be contracted during camping, hunting or hiking.** The human victim either t**ouches a dead infected rodent or is bitten by an infected flea.** The bacteria **invade the skin and are gobbled up by macrophages.** They continue to **reproduce intracellu- larly and within a week move to the nearest lymph nodes**, usually the **ONE SIDED INGUINAL NODES** ***_boubon is the Greek word for "groin")_***. The nodes swell like eggs and become **hot, red, and painful**. **Fever and headache** set in. The bacilli **invade the bloodstream, liver, lungs, and other** **organs**. Hemorrhages under the skin cause a **blackish discoloration**, leading people to call bubonic plague the "**Black Death."**
639
drug used to prevent Cerebro Vasospasm following Subarrachnoid HEMM
NIMODIPINE fill in
640
Deficiency of **Th17** cells due to **STAT3 mutation impaired recruitment of neutrophils to sites of infection.** **state sxs** **"FATED"** **what are the lab values?**
**Autosomal dominant hyper-IgE syndrome (Job syndrome)** **FATED:** coarse **F**acies, cold (noninflamed) staphylococcal **A**bscesses**!!!!!!\*\*\*\*\*\*** retained primary **T**eeth, INC Ig**E (blood eosinophilia)** **D**ermatologic problems *(eczema).* ***INC** IgE; **DEC** IFN-γ.* *pathogen: reason for high IGN is that theres **LOW Th1** (**pms do not respond to chemostaxis!!)** --\> **LOW IFN**--\> **NOT INHIBITION ON TH2-**-\> il4 wins--\> lots of class switching to i**gE.***
641
is an **overgrowth disorder** usually present at birth, *characterized by an increased risk of childhood cancer and certain congenital features.* **Wilms tumor** **macroglossia** (large tongue), macrosomia (above average birth weight and length), midline abdominal wall defects (**omphalocele/**exomphalos, umbilical hernia, ear creases or ear pits, **neonatal hypoglycemia** (low blood sugar after birth). **Hepatoblastoma**
**Beckwith-Wiedemann!**
642
marasmus, how diff from kwashikor
Total calorie malnutrition (no carbs no protein) resulting in emaciation (tissue and **muscle wasting,** loss of subcutaneous fat); +/– edema. **-anemia** **-dec cell med imm** **\*\*no edema, no ascites, no big belly!** **-NOT** Apathy, listlessness, poor appetite---\> GREAT PROGNOSIS! (2) **Loss of subcutaneous fat** • Due to a **decrease in leptin** stores in the adipose, which stimulates the hypothalamic-pituitary axis to **release cortisol, a lipolytic agent**
643
**HYPONATREMIA** **GI DISTURBANCEES (DIARRHEA)** **NEURO DISTURBANCES** **^^^should be all the clues you need to PICK THE BUG** but if not: AIR CONDITIONING + CRUISES (aerosol transmission by CONTAMINATED water source habitat) NO PERSON TO PERSON TRANSMISSION DETECTED BY SEEING ANTIGEN IN URINE **HOW DO YOU STAIN FOR THIS BUG?**
**LEGIONELLLLLLAAAA** **LEGIONAIRRES DISEASE** (ATYPICAL PMN + HIGH FEVER+ GI + NEURO SXS) STAIN WITH: **BCYE + IRON AND CYSTEINE** (USE **SILVER STAIN**!) **GRAM STAINS POORLY!**
644
name the most common disorders with an **X LINKED DOMINANT** mode of inheritance H-FAR
Hypophosphatemic rickets Fragile X syndrome Alport syndrome Rett Syndrome
645
tell me all about enterococci species. are they susceptible to penicillin G? what clinical diseases are they knows to cause? are they gram + or - are they cat (+/-)? PYR (+/-)? why are they an important cause of nosocomial infection?
Gram ⊕ cocci. Enterococci (E faecalis and E faecium) are normal colonic ora that are penicillin G resistant and cause UTI, biliary tract infections, and subacute endocarditis (following GI/GU procedures). Catalase ⊝, PYR ⊕, variable hemolysis. VRE (vancomycin-resistant enterococci) are an important cause of nosocomial infection.
646
In **young children** with **sudden cough** and **no feve**r **or URI symptoms,** the examiner should have a high index of suspicion for
foreign body aspiration.
647
how do you tell the difference clinically btw **RUBELLA** (TOGA) AND **RUBEOLA** (PARAMYXO)? bonus q: WHAT CAN YOU USE TO PREVENT A TYPE OF resp infection that paramyxo causes in infants?
**RUBELLA** (TOGA) presents with P**OST AURICULAR TENDER LAD!!!!!!** *BUT NOTTTTT RUBEOLOA* **[BOTH** START AT FACE WITH MACPAP RASH AND SPREAD TO TRUCK + EXTREMETIES!]\ TOGA---\> **CONGENITAL RUBELLA** (BLUBERRY MUFFIN BABY DUE TO DERMAL EXTRAMEDULLARY HEMATOPOIESIS) **PARAMYXO** HAS **F PROTEIN** THAT FORMS MULTINUCLEATED GIANT CELLS AND CAUSES PARAINFLUENZA (CROUP), MUMPS, RSV (causes resp tract infection in infacs (bronchiolitis, pmn)-----GIVE **PALVIZUMAB** *(MONOCOLONAL AB AGAINST F PROTEIN*) TO PREVENT PMN CAUSES BY RSV INFECTION IN PREMATURE BABIES
648
what cannot be digested if ACID (Hcl) is absent?
2. Digestion of **protein** a. Digestion begins in the s**tomach** (pepsin and acid) (1) Hydrochloric acid (HCl) cleaves pepsinogen into pepsin. (a) HCl is synthesized by parietal cells. (b) **If acid** is absent (e.g., **chronic atrophic gastritis**), protein cannot be digested.
649
Drugs that increase QT interval (predispose to Torsades de pointes)
Drug-induced long QT **(ABCDE)**: Anti**A**rrhythmics (class **IA, III**) Anti**B**iotics (eg, **macrolides**) Anti“**C**”ychotics (eg, **haloperidol**) Anti**D**epressants (eg, **TCAs**) Anti**E**metics (eg, **ondansetron**) **Ranolazine** (used to treat Angina refractory to other meds)
650
651
- BABY AT **3 WEEKS** - MOM WANTED TO TAKE THE **"NATURAL" ROUTE** SO HAD HIM AT HOME **NO NEONATAL VACINNES OR TREATMENTS POST BIRTH** BABY ALL THE SUDDEN HAS A **HUGE HEA**D **BULGING FONTANELLE** **ONLY BREASTFED** **EYES STUCK DOWN,** DONT TRACK UP FOUND TO HAVE AND **INTRACRANIAL HEMORRAGE** (ALSO GI BLEEDS, ETC) BABY WAS BORN AT FULL TERM NOT PREMIE WHY IS ALL THIS HAPPENING A. GERMINAL MATRIX FRAGILITY B. IMPAIRED CLOTTING FACTOR CARBOXYLATION
**B! IMPAIRED CLOTTING FACTOR CARBOXYLATION!**! BABY HAS IMMATURE GUT MOMS VIT K DOESNT CROSS PLACENTA AND IS BARELY IN BREAST MILK CLOTTING FACTORS CANT BE ACTIVATED (DUE TO LOW VIT K!) THEREFORE IMPAIRED CLOTTING! you're supposed to give babies vit K injection after birth to help this but this mom was stubborn! also it wouldnt be the germinal matric one bc these babies are **PREMIE and bleeding is INTRAVENTRICULAR!!!**
652
name two diseases where you have **_Primary Amenorrhea_** and and **absent uterus but external genetalia (female) are present** how about one disease where you **_Primary amenorrhea_**, **HAVE** a uterus & **atrophic** ovaries and + **no external genetalia**
**Androgen insensitivity Syndrome (X,Y)-**normal appearing female (but **no uterus or tubes** and a **rudimentary vagina)...the Y in the male allows MIF to be secreted so no UUF development (upper vag (lower from UG sinus), uterus, fallopian tubes)** **Mullerian Agenesis (Mayer-Rokitansky Kuster-Hauser Syndrome**)-have fully developed Secondary sexual characteristics bc ovaries are functional (but **VAGINAL AGENESIS: which is absent of an organ due to absent primordial tissue**) BUT **Turners** have Ovarian insufficiency (degenerated) so no Estrogen--\> **have uterus** but since **no (functional) ovaries**-\> no estrogen--\> **no secondary sex characteristics (breast dev or menstruation)**
653
“You cry, drool, and sweat on your ‘pilow.’ ” is a mneominc for WHAT direct acting cholinergic drug? **Potent stimulator of sweat, tears, and saliva** hint: its is **DOC for CLOSED angle glaucoma 911 cases** its **DOC for XEROSTOMIA seen in Sjrogren syndrome**
**PILOCARPINE!** **DIRECT M3 +** Contracts ciliary muscle of eye (open-angle glaucoma), pupillary sphincter (closed-angle glaucoma) resistant to AChE. “You cry, drool, and sweat on your ‘pilow.’ ” Use pilocarpine in emergencies—**very effective at opening meshwork into canal of Schlemm**
654
"**UNIFORMLY ENLARGED UNTERUS** (SOFT GLOBULAR UTERUS)" DX? PATHOGENESIS? PRESENTING SX?
**ADENOMYSOSIS** ENDOMETRIAL TISSUE CREEPING INTO MYOMETRIUM PAIN WITH MENSES (DYSMENORRHEA) HEAVY MENSTRUAL BLEEDING (MENORRHAGIA)
655
When spread of VAGINAL CARCINOMA to regional lymph nodes occurs, cancer from the **lower 2/3 of** vagina goes to....what LNs? cancer from the **upper 1/3 goes** to what LNs?
VAGINAL cancer from the **lower 2/3 of** vagina goes to: **inguinal nodes** cancer from the **upper 1/3 goes** to regional **iliac nodes**
656
what comes first in Coagulative Necrosis and what comes first in Liquefactive Necrosis? ## Footnote (Neutrophils release lysosomal enzymes that digest the tissue)--\> **1.enzymatic degradation first** **2.** **then proteins denature?** (Ischemia or infarction)--\> 1. **proteins denature** 2. **then enzymatic degradation**
(Neutrophils release lysosomal enzymes that digest the tissue)--\> **1.enzymatic degradation first** **2.** **then proteins denature?** **=====LIQUEFACTIVE!** (Ischemia or infarction)--\> 1. **proteins denature** 2. **then enzymatic degradation** **=====COAGULATIVE!** **[know this difference]**
657
what is FENOLDOPAM safe to use in pts with renal failure when they are having a HYPERTENSIVE 911? what about its MOA makes it safe?
it **maintaines renal blood flow** as it lowers BP by causing arterioloar DILATION of CORONARY, PERIHPHERAL, **RENAL** AND SPLANCHNIC arterioles.
658
strawberry hemangioma on kid do they progressively regress or get bigger rapidly and then regress?
GET BIGGER RAPIDLY AND THENNNNN REGRESS SPONTANEOUSLY BY PUBERTY (most cases by 7 y/0) (DO NOT PICK THE ANSWER THAT SIMPLY STATES THEY PROGRESSIVELY REGRESS)
659
what is the meaning of the following immature defense mechanism? ## Footnote **Identification**
Modeling behavior **after another person who is more powerfu**l (though not necessarily admired). **the child who got raped BECOMES THE RAPIST**
660
name the three murmurs that are "holosystolic" and identify which is the only one that INCREASES IN INTENSITY UPON INSPIRATION
at apex: MR at left sternal border (**\*\*\*\*TR\*\*\*\*** and VSD=harsh) TR increases in intensity upon inspiration
661
whats the MCC of LOBAR hemm in ELDER **WITHOUT HTN?**
**CEREBRAL AMYLOID ANGIOPATHY!!!**
662
which of following present with abd PAIN + bloody diarrhea which presents WITHOUT PAIN (only sometimes) intussiception volvulos meckels diverticulum henoch schnolem purpura
**meckels diverticulum** (usually pts are NOT IN PAIN; if yes its in the RLQ); presents in FIRST TWO YEARS OF LIFE! **intussiception: intermittent severe abdominal pain** often with **red “currant jelly” bloody stools**. associatied with recent viral infection, such as adenovirus **volvulos:** billious vomiting + abd pain +bloody diarhhea **henoch schnolem purpura (**crampy abd pain + palpable purpura + bloody diarrhea) **intermittent severe abdominal pain** often with **red “currant jelly” bloody stools**. associatied with recent viral infection, such as adenovirus
663
Small, tan to brown macule; darkens when exposed to sunlight Due to increased number of **melanosomes** (melanocytes are **no**t increased) what is being described?
**FRECKLES!!!!!!** (EPHELIS) Due to increased number of ***_melanosomes_*** (melanocytes are **no**t increased) vs: **VITILIGO (**Localized loss of skin pigmentation; Due to **autoimmune destruction of *_melanocytes_***
664
what presentations do the 3 groups of HPV strains have? 1-4--\> 6,11--\> 16,18--\>
1-4--\> **SKIN WARTS** [VERRUCA VULGARIS] 6,11--\> **GENITAL WARTS** [CONDYLOMATA ACUMINATA] 16,18--\> **CERVICAL, VAGINAL, VULVAR, ADN ANAL NEOPLASIA** (msm)
665
given a Helminth infection (US) bc they dont have you a travle history in the Q. which are the **TWO most common in the US**? whats their route of infection and treatment
**Enterobius vermicularis**, Intestinal infection causing anal pruritus (diagnosed by seeing egg A via the tape test) **Ascaris lumbricoides** (giant roundworm) Intestinal infection with possible obstruction at ileocecal valve-eggs visible in feces under microscope **bendazoles (all nematode round worms you treat with this!)**
666
20-year-old woman at **27 weeks' gestation** is admitted to the hospital because of a 12-hour history of **intense uterine** contractions occurring every 8 minutes. Her **membranes ruptured** 32 hours ago. Her temperature is 39.1°C **(102.4°F),** and pulse is 115/min. Physical examination shows **tenderness of the uterus.** Pelvic examination shows a closed cervix that is not effaced. The fetal heart rate is 210/min. Which of the following is the primary stimulus for her uterine muscle contractions? A) Decreased myometrial intracellular calcium B) Direct response to maternal hyperthermia C) Inflamed maternal decidua release of prostaglandin D) Maternal adrenocorticosteroid release E) Stressed fetal production and release of oxytocin why?
**C) Inflamed maternal decidua release of prostaglandin (she probs had an infection).** * **see beautiful (up to date) explanation below** Preterm premature rupture of membranes Genital tract infection is the single most common identifiable risk factor for PPROM. Three lines of epidemiologic evidence strongly support this association: (1) women with PPROM are significantly more likely than women with intact membranes to have pathogenic microorganisms in the amniotic fluid, (2) women with PPROM have a significantly higher rate of histologic chorioamnionitis than those who deliver preterm without PPROM, and (3) the frequency of PPROM is significantly higher in women with certain lower genital tract infections (particularly bacterial vaginosis) than in uninfected women. The association between bacterial colonization of the lower genital tract and PPROM is not surprising. Many of the microorganisms that colonize the lower genital tract have the capacity to produce phospholipases, which can stimulate the production of ***_prostaglandins_*** and thereby lead to the onset of uterine contractions. In addition, the host's immune response to bacterial invasion of the endocervix and/or fetal membranes leads to the production of multiple inflammatory mediators that can cause localized weakening of the fetal membranes and result in PPROM [3].
667
which gram negative bug am i describing? what disease are they mainly associated with? -they have **Carrier state with gallbladder colonization** **Primarily monocytes is its immune rxn** **need a High**—**large inoculum** required *because organism inactivated by gastric acids* only affects Humans (not animlas)
this is Salmonella typhi!!!! **Causes typhoid fever** (rose spots on abdomen, constipation, abdominal pain, fever); Both **Salmonella and Shigella are gram ⊝ rods,** **non-lactose fermenters, oxidase ⊝,** and **can invade the GI tract via M cells of Peyer patches.** Salmonella Typh **make H2S** just like salmonella but note! (shigella dont make it! and shigella does not do human to human spread, just spreads across cells! and shigellla is not motile bc it aint got flagella like the other two anddd bc its resistant to gastric acid you need a real SMALL inoculum to get sick from it. whomp.
668
669
Telangiectasias, recurrent epistaxis NOSEBLEEDS, skin discoloration, arteriovenous malformations, GI bleeding, hematuria WHAT DISEASE IS THIS? pattern of inheritance? NBME q: 45 yr old man with SOB on exertion x 6 months, **nosebleeds since adolescence**, 2 pics: **clubbing** + **hemorrhagic lesions in tongue,** inhaled albuterol doesn't improve his symptoms. Cause of symptoms? a- ASD b- HTN c- Pulmonary AV shunting d- reactive airway disease e- thromboembolism
**Osler-Weber-Rendu syndrome** **[Hereditary hemorrhagic telangiectasia]** **AD** **c**- Pulmonary AV shunting
670
Schaumann and asteroid bodies
Sarcoidosis
671
peripheral neuropathy, developmental delay, optic atrophy, **globoid cells.**
*Lysosomal storage disease* **Krabbe disease** Autosomal recessive lysosomal storage disease due to de ciency of galactocerebrosidase. **Buildup of** galactocerebroside and psychosine destroys myelin sheath
672
**Familial Mediteranean Fever** is an AR disease where neutrophils are messed up and can create an attack of acute inflammation (not due to an infection but bc the neutrophils just are active for no reason) and **IT CLASSICALLY EFFECT SEROSAL SURFACES** presents with fever + EG: in heart affects pericardium and presents as MI abdomen: presents as Acute appendicitis **what type of amyloidosis**
**AA (secondary)** **(not the AL from Ig Light chains)** Seen with **chronic in ammatory conditions** such as rheumatoid arthritis, IBD, spondyloarthropathy, **familial Mediterranean fever,** protracted infection. Fibrils composed of serum Amyloid A SAA---\> AA
673
1. **ADH antagonist** (member of tetracycline family). **SIADH TX.** 2. **Synthetic analog of aldosterone with little glucocorticoid effects. Mineralocorticoid replacement in 1° adrenal insuf ciency.** 3. Block action of ADH at V2-receptor. **SIADH Tx** 4. **Sensitizes Ca2+-sensing receptor (CaSR) in parathyroid gland to circulating Ca2+ PTH. TX: 1° or 2° hyperparathyroidism.**
1. **ADH antagonist** (member of tetracycline family). **SIADH TX.-------***Demeclocycline* 2. **Synthetic analog of aldosterone with little glucocorticoid effects.** Mineralocorticoid replacement in **1° adrenal insufficiency---***Fludrocortisone* 3. Block action of ADH at V2-receptor. **SIADH Tx-------***ADH antagonists (conivaptan, tolvaptan)* 4. **Sensitizes Ca2+-sensing receptor (CaSR) in parathyroid gland to circulating Ca2+ PTH. TX: 1° or 2° hyperparathyroidism-----***Cinacalcet*
674
675
Severe jaundice in neonate Absent UDP-glucuronosyltransferase. Presents early in life; patients die within a few years. Findings: jaundice, kernicterus (bilirubin deposition in brain), unconjugated bilirubin. what disease is this? there are 2 types, and one is less severe. how are each treated (diff treatments for each)
**Crigler-Najjar syndrome, type I** (MORE SEVERE) **Treatment:** plasmapheresis and phototherapy (non UV...simply makes BR soluble for excretion, doesnt conjugate it!) **Crigler-Najjar syndrome, type II (**less severe) **Treatment:** responds to **phenobarbital**, which INC liver enzyme synthesis.
676
677
lady has a skin lesion in her upper eyelid biosposed and **lipid laden macrophages** are found in the superficial dermis. what are these skin conditions associated with?
diseases with **hyperCHOLESTEROL!!!** **XANTHELESMAS!!!!!** eg: dyslipidemias 1 or 2a also **chronic CHOLESTATIC DISEASES LIIIKKKEE** **PRIMARY BILIARY CIRRHOSIS!!!!!!!!** ps: you would see hyperlipidemia in HYPOthyroidims not hyper
678
epithelial cells covered with GRAM VARIABLE RODS ARE AKA?
CLUE CELLS IN BACTERIAL VAGINOSIS (pH\>4.5) +ve whiff test (amine odor with KOF)
679
i got this wrong. ansewr is actually C. when in a womans cycle is estrogen about the highest and progestrone is low? day: 1, 6, 12, 18 or 26?
Between approximately days **9- 13, the estradiol level dramatically rises.** At a certain point, it no longer has an inhibitory effect on LH and FSH, but paradoxically a stimulatory effect. The oocyte exits from the follicle, the new corpus luteum is stimulated by LH to secrete considerable progesterone, and the luteal phase continues. However, the progesterone level would be much below that of estradiol UNTIL after the LH surge. **Answer= C**
680
**Fibrous bands (Ladd bands) are seen in...**
**Malrotation** **Anomaly of midgut rotation** during fetal development--\> improper positioning of bowel, formation of fibrous bands (Ladd bands). Can lead to volvulus, duodenal obstruction
681
a "thrill" accompanies what type of murmur usually and where is this murmur heard the loudest?
VSD harsh holoSYStolic thrill loudest at tricuspid area actually VSD in Tet of Fallot
682
are the synaptic conc of Ach decreased in MG?
NOOO! but they are dec in Botulism and Lambert eaton
683
what is the **first line treatment** for the psych disorder in which **VENTRICULOMEGALY** is characteristic and what are the main AE of this med?
**Atypical** antipsychotics **Schizophrenia**—treats **BOTH** both positive **and** negative symptoms. **Risperidone**—**hyperprolactinemia--\>** (**amenorrhea, galactorrhea, gynecomastia)**. **D2 antagonists;**
684
look at the picture. note the "cerebriform nuclei" in the CD T cell DIAGNOSIS?
**Mycosis fungoides/ Sézary syndrome**
685
2,3 BPG normally forms an ionic bond with the beta subunits of DEOXYGENATED HbA so that no new o2 can bind and unloading occurs, if someonen has a mutation that decreases this affinity, (eg: the Lysine residue (+ve charge) is switching to Methionine to that the binding of 2,3 bpg cant happen as strongly, which type of Hb is this most similar to? \*note usually the 2,3BPG biniding pocket on these beta chains of normal adult Hb is conveniently made of (his and Lystine! +ve guys!) HbC HF HbAIc
HbF!!!! in order to be super selfish and extract as much O2 as possible from mom, it has GAMMA instead of beta chains and, unlike beta chains these gamma chains DO NOT bind 2,bpg (which is -ve charge) bc instead of Histinidine (+ve) is has SERINE!
686
in CT at level L2 of abdomen is the IVC on the left or right side of the patient?
RIGHT! (it si medial to the duodenum adn anterior to the right side of the verterbral body) the simlar but slightly smaller round structure to the left of the IVS is the abdominal aorta (which bifurcates at L4)
687
which brain tumor can produce **EPO** and therefore **Secondary Polycythemia** is a complication
Hemangioblastoma Most often **cerebellar** . Associated with **von Hippel-Lindau syndrome** when found with **retinal angiomas**. Can produce **erythropoietin--\> 2° polycythemia.** HISTO: Closely arranged, thin-walled capillaries with minimal intervening parenchyma
688
what are the coronary arteries like in HCM?
**freakin JUST FINE AND NORMAL!!!!!!!!!!!!!!**
689
what does the suprachiasmatic nucleus regulate? how does it work? which takes longer to recover from? eastward or westward travel?
**CIRCADIAN RHYTHMA!** info comes in from (photosensitive ganglion cells) in the retina, **SCN** relays info to **hypothal nuclei and pineal gland** to modulate body temp, and prod of hormones like **cortisol** and **melatonin (induces sleep)** **eastward travel harder to get over jetlag**
690
which vasculitis' give you +ve C-ANCA
just **Wegeners** (granulomatosis with Polyangitis)
691
are **oral apthous ulcers** moreso associated with CD or UC?
CD!!!!!!!!
692
what Three bugs can cause a "blueberry muffin rash" hint: both are TORCH infections
1. Toxoplasma gondii Classic triad in BABY: chorioretinitis, hydrocephalus, and intracranial calci cations, +/− “blueberry muf n” rash in mom: ASYMPTOMATIC 2.Rubella (Toga) Classic triad in BABY: abnormalities of eye (cataract) and ear (deafness) and congenital heart disease (PDA); ± “blueberry muf n” rash. in MOM: Rash, lymphadenopathy, polyarthritis, polyarthralgia 3. CMV Hearing loss, seizures, petechial rash, “blueberry muf n” rash, periventricular calci cations
693
Mites that burrow into the **stratum corneum a**nd cause pruritis. Causes **serpiginous burrows (lines)** in **webspace** of **hands and feet**
**Scabies** Common in children, crowded populations (jails, nursing homes); transmission through fomites. Treatment: **permethrin cream**, washing/drying all clothing/bedding, **treat close contacts.** | (Sarcoptes scabiei)
694
euvolemic hyponatremia with continued Na+ excretion seen in
**SIADH** classic association too much adh normal bp anp and bnp to the rescue
695
if someone gets **stabbed in the RUQ** and they are **bleeding a ton** a doc will do the **pringle manueve**r t**o distinguish the source of the RUG bleeding** _2 questions_ 1. what does the pringle manuever entail? 2. lets say the pringle manuever is done and there is STILL a ton of bleeding (and you see a brick non pulsatile mass eminating from the back area of liver) WHAT COULD IT BE? MOST COMMONLYYY
1. pringle maneuver is occulsion of the **PORTAL TRIAD** in the **HEPATODUODENAL LIG** **1. Portal Vein** **2. Hepatic Artery** **3. Common bile duct** 2. it would have to be a VEIN bc its NON PULSATILE FIRST OFFFFF. and the most commonly causes of RUQ bleed if you ruled out anything involving contents of portral triad = **IVC !!!!!!!!** or _HEPATIC VEIN_
696
Aromatic amines (eg, **benzidine, 2-naphthylamine**) are found where and what kind of cancer does it cause? how about **arsenic**
Aromatic amines (eg, **benzidine, 2-naphthylamine**)---found in CIGARETTE SMOKE---\> **Transitional cell carcinoma** **A**rsenic---\> **L**ung cancer * *A**ngiosarcoma (LIVER) * *S**quamous cell carcinoma (SKIN) for arsenic think: **ALAS**
697
* Reversibly **inhibits dihydroorotate dehydrogenase----\> preventing pyrimidine synthesis**. * Suppresses T-cell proliferation. * used to treat: Rheumatoid arthritis, psoriatic arthritis. * AE: Diarrhea, hypertension, **hepatotoxicity, teratogenicity.** ## Footnote **what drug?**
**Leflunomide** **(look at the little pathway)**
698
Dont make this mistake again. ASIDE FROM USING IMAGING TO DIFF PILOCYTIC ASTROCYTOMA (cystic and solid) AND MEDULLOBLASTOMA (just solid) IN A MID....YOU CAN USE HISTO! HOW
rosental corskrew fibers eosinophii are pilocytic astrocytoma and a small blue cell PNET homer wright rosette are Medulloblastoma!!!!!!!!!!!!!
699
Billious emesis in a neonate is a sign of OBSTRUCTION below _which_ part of the duodenum (parts 1-4) what are **3 solid differentials** in an infant for such a thing? do you associate abdomincal distention with more DISTAL obstuction or more proximal and how about billious vomiting, more distal or proximal?
below part 2! 1. intestinal atresia (duodenal if below second part) or jej/ileum 2. midgut volvulus (911 situation) 3. intestinal stenosis proximal is more billious if more distal = moreso see abdominal distention (eg: colonic atresia or hirshsprungs)
700
at what level do the renal veins join the IVC? at what level do the common iliac veins merge to become the IVC
L1/L2 L4
701
Autosomal dominant Mutation in TSCl (hamartin) and TSC2 (tuberin) genes, which are tumor suppressor proteins
**Tuberous Sclerosis Complex** Autosomal dominant Mutation in TSCl (hamartin) and TSC2 (tuberin) genes, which are tumor suppressor proteins Multiple hamartomas Cortical tubers Renal angiomyolipomas Cardiac rhabdomyomas Pulmonary hamartomas
702
ALL ANTACIDS cause what electrolyte disturbance commonly?
All can cause **hypokalemia.**
703
WHAT ORGAN IS SAFEST IF THERE WERE SUDDEN BLOOD LOSS
LIVER NOT SPLEE, OR HEART OR BRAIN OR KIDNEY THE LIVER (WITH ONE NOTABLE EXCEPTION MENTIONED BELOW)
704
contrast the amount of collagen synthesis in a hypertrophic vs keloid scar and also the organization of this collagen
hypertrophic scar: amount of collagen synthesis in a hypertrophic is INCREASED keloid scar: amount of collagen synthesis is SUPER SUPER INCREASES the organization of this collagen hypertrophic scar=parallel keloid scar=disorganzied (more common in dark skinned people)
705
saddle nose + oliguria + red cell casts + treat with cyclophosphamide/corticosteroids
WEGENERS! glomerulonephritis--\> oliguria performation of nasopharyn--\> saddle nose (also seen in congenital syphilis) Granulomatosis with Polyangitis PR3/c-ANCA +ve
706
Charcot triad of cholangitis?
Charcot triad of cholangitis: ƒ Jaundice ƒ Fever ƒ RUQ pain
707
In, for example, **Rheumatic Heart Disese/Fever,** what are the **hemodynamic changes** seen in Left heart, Right heart Pulmonary circuits if **only the Mitral Valve is involved**...... How about is the **Aortic valve** is involved? What now.
**ISOLATED MITRAL STENOSIS-**-\> inc LA diastolic P--\> inc PCWP, Pulmonary HTN, Dec Pulm Vascular Compliance, RV dilatation--\> functional Tricuspid regurg! \*\*LEAVES **LV DIASTOLIC PRESSURE** ALONE (or even decreased when stenosis is severe)! if **Aortic valve** is involved--\> **LV DIASTOLIC PRESSURE IS INCREASED**
708
what hematological disorder is characterized by atypical erythroblasts with **granules of iron accumulated in the mitonchondria around the nucleus**? WHAT STAIN IS THE MOST ACCURATE?
**SIDEROBLASTIC ANEMIA** note: although x linked, 1/3 of women have it due to skewed x inactivation **PRUSSIAN BLUE!** THIS IS A MICROCYTIC ANEMIA
709
**Delayed wound healing**, **hypogonadism,** **decreased adult hair-alopecia** (axillary, facial, pubic) **dysgeusia** (foul, salty, rancid, or metallic taste sensation will persist in the mouth) **anosmia** **acrodermatitis enteropathica** (can be acquired (nutritional deficiency, dec uptake, inc loss) or genetic (AR) due to impaired uptake-- but both present similiarly *(eczema, xerosis (dry, scaling skin), seborrheic **dermatitis*** **May predispose to alcoholic cirrhosis.** what type of deficiency would a person who presented like this have? why would they have IMPAIRED WOUNT HEALING?
**ZINC DEFICIENCY** **in the last step of wound healing called "Remodeling" (1 week–6+ months after wound)** **Fibroblasts Type III collagen replaced by type I collagen...this is mediated by COLLAGENASE WHICH USES ZINC AS A CO FACTOR!** note: zinc can also help to treat **Wilson's disease**
710
a crytogenic stroke is its a stroke that has an undefined origin like its not cardioembolic, etc **what are some common causes of this?** eg: someone young (or of any age) alls the sudden has sudden onset unilateral weakness and difficulty speaking (eg: acute LEFT FRONTAL LOBE infarction)
**PFO = HIGHLY ASSOCIATED WITH PARADOX EMBOLISM** (incomplete fusion of the ATRIAL septum primum and secundum) \*\*\*note these are usually closed but if a patient does something to INC pressure in RA LIKE RELEASING a valslava (which itself normally dec preload and afterload), it can caused flow of blood from the RA--\>\> LA and then cause a paradoxical embolism leading to a cryptogenic stroke! ASD
711
how can rotavirus affect disaccharidase activity?
Brush border intestinal enzymes **(disaccharidases)** (1) Lactase hydrolyzes lactose to give galactose and glucose, maltase hydrolyzes maltose to produce two glucoses, and sucrase hydrolyzes sucrose to form fructose and glucose. (2) **Disaccharidase**s produce **glucose, galactose, and fructose.** (a) In certain types of diarrhea (e.g., **Rotavirus)**, the **brush border is temporarily** * *destroyed** and **disaccharidases cannot hydrolyze disaccharides.** (b) People with lactase deficiency cannot hydrolyze lactose in dairy products.
712
arteries and veins which one would clinically be PULSATILE vs NON PULSATILE
veins are NON PULSATILE arteries are PULSATILE
713
what enzyme does **e coli** make that **breaks lactose into glucose and galactose via lac operon**?
**b-galactocidase** **also makes permease and transacetylase enzymes**
714
slow growing papule with ULCERATION AND ROLLED BORDERS makes you think of what cancer?
BASAL CELL CA NOTE: picture
715
Presents with **dermatitis (necrolytic migratory erythema)**, diabetes (**hyperglycemia**), **DVT**, **declining weight**, **depression**. Treatment: octreotide, surgery.
**GLUCAGONOMA!!**
716
what should be suspected in young adults with **recurrent oral aphthous ulcers**, unexplained ocular findings, or genital ulcers.
**Behcet's syndrome** is a multisystem, r**elapsing**, **chronic vasculitic** (due to **immune complex**) disorder with prominent mucosal inflammation. Common manifestations include **recurrent oral ulcers**, ocular inflammation, **genital ulcers, and skin lesions**. The **most serious manifestations are blindness,** neurologic or GI manifestations, **venous thromboses,** and **arterial aneurysms.** Diagnosis is clinical, using international criteria
717
disorder characterized by **short stature, predisposition to the development of cancer and genomic instability.** **-**caused by **mutations in the BLM gene** leading to **mutated *DNA HELICASE MUTATION*** characterized by **genome instability.** The most prominent features include short stature and a **rash on the face that develops early in life when exposed to the sun (PHOTOSENSITIVE)**. The skin rash is erythematous, **telangiectatic**, infiltrated, and scaly, have a **high-pitched voice** and distinctive facial features including a **long, narrow face**; a small lower jaw; and **prominent nose and ears.** Other features can include learning disabilities, an increased risk of diabetes, chronic obstructive pulmonary disease (COPD),
**BLOOM SYNDROME**
718
cerebellar hemangioblastomas and retinal angiomas. THIS COMBO IS NONE OTHER THAN
**Von Hippel-Lindau Disease** Von Hippel-Lindau disease (VHL) is a rare hereditary neurocutaneous disorder characterized by tumors in multiple organs.
719
**Benign** epithelial cell tumor **arising from collecting ducts** **Large eosinophilic** cells **with abundant mitochondria** **without** *perinuclear clearing* NOTE!!!! THIS IS ONE OF THE BIG WAYS TO DIFF IT FROM RCC (vs chromophobe renal cell carcinoma WHICH DOES HAVE PERINUCLEAR CLEARING). Presents with painless hematuria, FLank pain, abdominal mass. **Often resected to exclude malignancy (eg, renal cell carcinoma). BC SO SIMILAR ON HISTO**
**Renal oncocytoma**
720