UWorld second half Flashcards

(487 cards)

1
Q

Developmental field defect

A

An initial embryonic disturbance that leads to multiple malformations by disrupting the development of adjacent tissues

Holopsoencephaly (due to incomete division of the forebrain) is an example of this where it is likely caused by a combination of genetic (trisomy 13, sonic hedgehog gene defects) and environmental (maternal ETOH use)

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2
Q

Treatment for bulemia nervosa

A

SSRI (fluoxetine)

Nutritional rehab

CBT

*Remember Bupriprion is CONTRAINDICATED in bulemia pts due to risk of seizure*

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3
Q

Xeroderma Pigmentosum

A

Rare AR disorder caused by ineffective nucleotide excision repair of damaged DNA by UV light

Leads to the accumulation of pyrimidine nucleotides.

These pts appear normal at birth but then get progressively worse when exposed to the sun. Skin malignancies such as melanoma can present as early as 5

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4
Q

What happens to systemic vascular resistance during exercise?

A

DECREASES

Due to arterial vasodilation in active muscles, which is mediated by local release of Adenosine, K, ATP, CO2, Lactate

The things that INCREASE are:

CO, LV end diastolic pressure (due to increase in LV filling), Pulm artery systolic pressure, systemic systolic pressure

During exercise, there is a splanchnic vasoconstriction but the vasodilation at the skeletal muscles make up for this and see a modest increase in mean blood pressure

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5
Q

Lamotrogine mechansim, use and side effect

A

Used for partial and generalized seizures, blocks voltage gated Na channels

Watch out for STEVEN JOHNSON SYNDROME! Also toxic epidermal necrolysis is rare but fatal side effect

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6
Q

What muscle causes the proximal half of the clavicle to rise superiorly upon clavicular fracture?

A

Sternocleidomastoid muscle pulls clavicle superiorly and posteriorly.

The weight of the arm and the pec major pull the lateral fragment laterally and inferiorly

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7
Q

How can an atherosclerotic plaque that occuludes the artery 100% not cause downstream necrosis in the heart?

A

It grows slowly and the heart has time to adapt with arterial collaterals around the point of occlusion

*The major determinant on whether or not an atherosclerotic plaque will cause ischemic myocardial injury is the rate at which it grows

SLOW –> allow for collaterals to develop

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8
Q

Transformation of DNA esp in regards to Strep Pneumo

A

Strep pneumo strains that do not have a capsule are not virulent, they do however, have the ability to obtain new genetic material from the environment that is released following the death and lysis of bacterial cells. This process is known as TRANSFORMATION. This is a process by which it can gain virulence

Other bacteria that can do it:

Haemphilus, Neisseria, Bacillus, Streptococcus

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9
Q

BRAF V600E val–>glutamic acid mutation is common in what disease and what is a great drug to treat this??

A

Seen in 40-60% of pts with melanoma

BRAF is an oncogene that is a protein kinase involved in activating the signaling pathway of melanocyte proliferation

Vemurafinib is a small molecule inhibitor of BRAF oncogene + melanoma

VEmuRAFenib is for V600E-mutated BRAF inhibiton

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10
Q

C1 esterase inhibitor deficiency

A

Causes hereditary angioedema due to unregulated activation of kallikrinin –> increased bradykinin (a potent vasodilator that causes increased vascular permeability)

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11
Q

Thrombocytopenic Thrombotic purpura- hemolytic uremic syndrome (TTP-HUS)

A

1. Platelet activation

2. Diffuse microvascular thrombosis

3. Microangiopathic hemolyitc anemia with schistocytes

4. Thrombocytopenia

TTP is almost always characterized by a normal PTT (unlike in DIC)

Would present with platelt rich thrombi in the glomeruli and arterioles

Pt presentation has pentad:

fever, neurologic symptoms, renal failure, anemia, thrombocytopenia

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12
Q

Bethanechol

A

Muscarinic cholinergic agonist that is used to improve bladder emptying in post-surgical pts

Bethany, call me to activate you Bowels and Bladder

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13
Q

1 std dev

2 std dev

3 std dev

A

1: 68%
2: 95%
3: 99.7%

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14
Q

Dofelitide (cardiac)

A

Class III antiarrhymic drug that blocks potassium channels and inhibit outward potassium during phase 3 of the cardiac action potential thereby prolonging repolariazation

No effect on AP conduction velocity, prolonges AP duration

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15
Q

Radial traction of the lungs is increased/decreased with emphysema and pulmonary fibroris

A

Emphysema –> decreased traction

Pulmonary fibrosis –> increased traction

Radial traction=outward pulling by the surrounding fibrotic tissue. This results in decreased airflow resistance leading to supranormal expiratory flow rates (which makes sense with restrictive lung dieases being a problem getting air in NOT getting air out)

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16
Q

Most commonly injured leg nerve and cannot evert foot properly

A

Common peroneal nerve

Fibular neck fractures or prolonged casting or immobilization can injure this nerve. Present with plantarflexed and inverted posture due to paralsys of the peroneus longus and peroneus brevis (eversion muscles)

Presents as “foot drop” with compensated “foot slap”

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17
Q

Quick way to remember if disease is AR or AD

A

If cause defect in enzyme –> autosomal recessive

If cause defect in structural protein –> autosomal dominant

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18
Q

What are target cells seen in?

HALT said the hunter to his target”

A

H: Hbc

A: Asplenia

L: Liver Disease

T: Thalassemia

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19
Q
A
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20
Q

Symptoms of LEAD poisoning

A

L: Lead Lines on gingivae (burton lines) and on metaphyses of long bones

E: encephalopathy and erythrocyte basophilic stippling

A: abdominal colic and sideroblastic anemia

D: drops. Wrist and foot drops. Also treat with Dimercaprol and EDTA (succimer used for chelation for kids…sucks to be a kid who eats lead)

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21
Q

Pt is in heart failure with fluid overload, what is going to happen to their:

BNP

Sympathetic System

RAAS

A

BNP INCREASED: released in response to stretch (fluid overload). It is often used to help diagnose congestive heart failure exacerbation

Sympathetic System is INCREASED due to a perceived initial drop in blood pressure, which triggers compensatory neurohurmoral stimulation directed at maintaining blood pressure and tissue perfusion

RAAS system is also INCREASED for the same reason as above to improve cardiac output by increasing chronotropy and inotropy, expanding the ECF comparment and promoting atrial and venous vasoconstriction

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22
Q

What does von Willebrand factor actually do?

A

After endothelial damage, vWF binds GP1b receptors on the platelet membrane and mediates platelet aggregation and adhesion to subendothelial collagen

Deficiency presents as gingival bleeding often

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25
Q

HbA2 level grealty increased 7.5% (normal 1-3), is indicative of what?

A

Beta-thalassemia trait. HbA2 is increased in minor and greatly increased in major

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26
Q

What nerves travel through the jugular foramen?

A

9,10,11, jugular vein

Lesion here can cause Jugular foramen syndrome. Sx related to nerves involved (loss taste, loss gag reflex, dysphagia, hoarsness, deviation uvula, SCM and trapezius weakness)

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27
Indications for clozapine
**Treatment resistent schizophrenia or schizophrenia associated with suicidal ideation** Remember side effect of agranulocytosis need to **monitor clozely with clozapine**
28
Halothane induced hepatotoxicity, what is it and what are some of the lab findings
Predominantly cause a hepatocelluar pattern of liver injury. Can range from mild to fulminent hepatitis with 50% mortality rate. Liver looks very atrophied on exam at autopsy Lab: **elevated serum aminotransferases, prolonged PT (due to failure of production of factor VII),** leukocytosis, eosinophilia
29
Urine turns black. what is it?
**Alkaptonuria** Problem with **Tyrosine --\> Fumarate** **No Homogentisic Acid Dioxygenase** **Ochronosis=black urine** **(could be acute intermittent porphyria too if turned port wine colored --\> black overnight)**
30
Celiac Disease levels of: PTH Ca Phosphate
Celiac patients have excessive diarrhea that is fatty. This means a **decrease in ADEK vitamins**. Thus, these pts will have a **decrease in Vitamin D**. If D is low, then Ca and Phosphate are not being brought in from the gut. As a compensation mechansim, PTH will be high. **PTH: HIGH** **Ca: LOW** **Phosphate: LOW**
31
Immune system aspects that are fighting: Systemic Candidiasis (candidemia) Local Candidiasis
**Systemic: Neutrophils** prevent the systemic spread of candidiasis. Disseminiated Candidiaisis is more common in pts who are neutropenic **Local: T Lymphocytes** are important for prevention of superficial candidial infections (oral/esophageal) Thus, HIV pts suffer from local candida infections but rarely suffer from systemic infections. If they are neutropenic for whatever reason they are at risk for both local and systemic
32
Free wall rupture of ventricle happens when post MI
5-14 days post MI
33
Combining Gancyclovir with TMP-SMX puts patient at risk for what?
**Bone marrow suppression** Gancyclovir itself puts pts at risk for **neutropenia**, anemia, thrombocytopenia, impaired renal fxn. Used for cytomegalovirus in HIV pts often
34
most common cause of viral meningitis?
**Enterovirus** (cocksackie, echo, polio) responsible for \>90% of cases CSF: normal glucose, marginally elevated protein, pleocytosis with a **LYMPHOCYTIC** predominance. This pattern suggests aseptic meningitis, which is viral in etiology
35
What is the consequence if start warfarin without a heparin bridge?
Warfarin blocks epoxide reductase so you cannot gamma carboxylate 2,7,9,10,C,S factors. It turns out that **C and S have short half lives (VII is the shortest** and reason why you can measure PT and not PTT) of any of these factors and thus run out first. These are inhibitory factors, so if they are gone you are in a **hypercoagulable** state (WHEN YOU WANT TO BE ANTICOAGULATED). This can lead to **warfarin induced skin necrosis** Tx: fresh frozen plasma, protein C concentrate
36
Pt has been applying cream for weeks and starts to get this erythematous rash with blisters, ulcers, and weeping drainage. What is this? How is it characterized histologically?
This is a classic example of eczema, specifically, the patient has an acute allergic contact dermatitis that is mediated by a type 4 hypersensitivity reaction to an antigen on the skin surface (cream). The antigen was taken up and presented to the Langerhan cells and presented to CD4 cells in the lymph nodes Histologically, this is characterized by **spongiosis**, accumulation of edema fluid in the intracellular spaces of the epidermis
37
Thickening of the statum spinosus --\> Thickening of stratum corneum --\> Thickening of stratum granulosum --\> Dyskeratosis --\> Separation of epidermal cells --\>
Stratum spinosum --\> acanthosis Stratum corneum --\> hyperkeratosis (**psoriasis, calluses)** Stratum granulosum --\> hypergranulosis (**seen in lichen planus**) Dyskeratosis --\> premature keratinization of individual keratinocytes below the stratum granulosum **(squamous cell carcinoma)** Separation epidermal cells --\> acantholysis **(pemphigus vulgaris)**
38
Spinal stenosis, and leads to hypertrophy of what spinal ligament?
Results in lower extremity pain, numbness/parasthesias Symptoms often worsen when standing up tall and get better when leaning forward (shopping cart) Degenerative arthritis of the spine is the most common type of spinal stenosis. The disc degenerates and begins to portrude and lose height. This places a large load on the POSTERIOR aspect of the spinal column leading to hypertrophy of the **ligamentum flavum**
39
Risk factors for biliary sludge
Pregnancy Rapid weight loss High spinal cord injuries Prolonged TPN Octreotide Biliary stasis --\> bile concentration --\> bile ppt --\> biliary sludge (a precursor to stone formation)
40
Holosystolic murmur heard over the cardiac apex?
**Mitral valve regurgitation** Often accompanied by S3 (increased LV filling rate during mid diastole) Often times treatment with a diuretic to decrease total fluid decreases the S3 (or makes it go away entirely). This happens via a decrease in LV preload and therefore decrease in LV EDV
41
Best antibiotic to use for aspiration pneumonia
Clindamycin
42
Pt has a combo of absent seizures and tonic-clonic, what is a good med to give them?
**Valproate good for BOTH** Ethosuximide would be good for the absent seizure but NOT for the tonic-clonic Phenytoin would be good for tonic-clonic but NOT the absent seizures Carbamezapine is drug of choice for complex partial seizures and is also effective in generalized tonic-clonic seizures but has side effect of agranulocytosis and aplastic anemia
43
What is the difference between splenic congestion and splenic fibrosis/atrophy in regards to a sickle cell pt?
**Splenic congestion** --\> can be seen in infants (10-15% mortality), which occurs due to vasoocclusion and splenic pooling of erythrocytes. Would see rapidly enlarging spleen and hypovolemic shock **Fibrosis/atrophy**
44
What type of anemias are sickle cell pts predisposed to and why
**1. Macrocytic megaloblastic anemia due to folic acid** deficiency due to increased erythrocyte turnover **2. Extramedullary erythropoesis** can occur in SCD and lead to elevated MCV (as the RBCs are larger). Also, this tends to make only a slightly elevated MCV \*\*The reticulocyte index would help decide which is happening. If the index is low, it is folic acid, if high, then extramedullary\*
45
Major aspects of a GPCR (3) and the AA associated with them
**1. extracellular domain:** respinsible for ligand binding **2. transmembrane domain:** made up on **NON-POLAR HYDROPHOBIC AA (alanine, valine** leucine**, isoleucine,** phenylalanine**,** tryptophan**,** methionine**,** proline**,** glycine). The hydrophobic aspect is arranged OUTWARD to **anchor** the transmembrane region in the phospholipid bilayer **3. Intracellular domain:** coupled with heterotrimeric G-proteins
46
Single red reflex? Assocaited with developing what OTHER type of tumor?
Retinoblastoma presents with white pupillary reflex in children \<5 years old **Familial retinoblastoma occurs due to a germline mutation in the Rb gene on chr 13** (remember that an active Rb does not allow a cell to go from G1 --\>S via inhibiting EF2, inactive Rb permits cell division) Likely to develop **osteosarcoma** later in life
47
Hemolytic disease of the newborn
**=erythroblastosis fetalis** Results in **destruction of fetal red blood cells by maternal antibodies directed against fetal erythrocyte antigens.** These antibodies are IgG antibodies (the only cass that can cross the placenta G=go) **1st preg:** Rh-mom/Rh+dad Rh+ kid --\> these fetal Rh antibodies enter the maternal blood stream and the mother generates anti-D antibodies **2nd preg:** Rh-mom/Rh+dad Rh+kiddo. Now the Anti-Rh-D antibodies cross the placenta and cause lysis of fetal RBC. This results in a **positive coombs test,** profound **anemia**, **jaundice** (possibly leading to kernicterus) and generalized edema (hydrops fetalis due to accumulation of interstitial fluid). Also see **extramedullary hematopoeisis** in liver, spleen and other tissue
48
Example of true diverticulum vs a pseudodiverticulum
**True:** Meckel's, Normal appendix. Contain mucosa, submucosa, and muscularis **Pseudo:** Zenker, diverticula. Contain ONLY mucosa and submucosa
49
Marfan syndrome mutation and cardiac manifestations
Genetic defect in glycoprotein **fibrillin-1,** resulting in abnormal skeleton **Cardiac:** see **mitral valve prolapse** and **cystic medial degeneration of the aorta** --\> resulting in aneurysmal dilation in 70% of cases. This can cause death if untreated and is the most common cause of death in Marfan pts
50
Child squatting on the playground has what?
**Tetrology of Fallot (VSD, overriding aorta, pulmonary artery stenosis, RVH**) Due to abnormal neural crest cell migration leads to anterior and cephalad deviation of the infundibular septum Squatting increases systemic vascular resistance without changing pulmonary vascular resistance which increases the ratio of SVR:PVR. This decreases Right to left shunting, thereby increasing pulmonary blood flow and improving oxygenation status
51
Take opioids and get severe URQ abd pain, what is mechanism?
Can cause **contraction of smooth muscle cells in the sphincter of oddi**, leading to spasm and an increase in common bile duct pressure. Although uncommon, pressures can increase and cause biliary colic. Management includes discontinuing of opioids
52
Two fates of pyruvate
Can enter into TCA cycle and make energy OR can enter into gluconeogenesis and make glucose Know that Acetyl CoA acts to increase the opposite pathway and stop its own pathway (negative feedback). This prevents the cell from being energy starved
53
What is cord factor's role in TB?
Presence of **cord factor correlates with virulence**. Mycobacteria that do NOT have cord factor are NOT able to cause disease It is responsible for inactivating neutrophils, damaging mitochondria, and inducing release of TNF-Alpha
54
How do you treat a specific phobia like elevators, heights, birds, injections...
Behavioral therapy is the treatment of choice for specific phobia Can use short acting benzos acutely Beta blocker is used to treat performance anxiety but is NOT indicated for phobias
55
What test do you use to screen for fecal fat?
Sudan III Stain
56
Refamiliarize with the steps of the urea cycle
57
Golgi tendon
Feedback stystem that monitors and maintains muscle force. GTO are exquisetly sensitive to increases in muscle tension but are insensitive to passive stretch Innervated by group Ib sensory axons Connected in SERIES with the extrafusal skeletal muscle fibers **Negative feedback system that regulates and maintains muscle tension**
58
How do you increase a HOCM murmur?
**sudden standing, nitro, valsalva (thus all 3 are contraindicated in these pts)** These pts have LV outflow tract obstruction (25% of HCM pts) Basically, **mechanisms that decrease preload (venous return) or decrease afterload, reduce LV chamber size**, which decreases the separation between the mitral valve and the interventricular septum, increasing obstruction **These actions INCREASE THE MURMUR**
59
Myocardial hibernation
Refers to a sta**te of chronic myocardial ischemia in which function and metabolism are decreased to match a concomminant reduction in coronary blood flow.** This new equilibrium prevents necrosis This leads to decreased contractility and LV systolic dysfunction Upon revascularization, the heart muscle can leave its hibernation state and improve contractility and LV function
60
Erythema multiforme | (think stamps on post man arm)
Target lesions with a dusky central area This is a cell-mediated immune process that is brought on by CD-8 T cells Most frequently associated with HSV and mycoplasma but can be associated with sulfa drugs, B-lactams, phenytoin \*\*not to be confused with erythema MIGRANS which is seen in lymes disease\*\*
61
wrinkles around eyes with old age are characterized by a decrease in what?
**wrinkles = decrease in collagen fibril production** UVA damage causes photoaging via production of ROS leading to a decrease in collagen fibril production along with upregulation of MMPs Photoaging may be visable by age 30-35 and results in thinning of the epidermis There is **INCREASED CROSSLINKING of collagen**
62
Blood/Gas partitioning coefficient
**poorly soluble gas has decreased blood/gas coefficient** **highly soluble gas has a high blood/gas coefficient** **Nitrous oxide is poorly soluble gas with blood/gas coefficient of 0.47** (meaning that it saturates quickly, leading to a fast rise in partial pressure, this also enters the brain quickly. The speed of anesthetic induction is equal to the speed at which the brain tissue takes up the agent **WANT A POORLY SOLUBLE DRUG**
63
Pathophys of Tetralogy of Fallot (why does it occur)
**Abnormal neural crest cell migration leads to anterior and cephalad deviation of the infundibular septum** resulting in malaligned VSD and overriding aorta
64
Pathophys of Left sided heart failure causing pulmonary HTN
As a result of the LV hypertrophy and decreased LV diastolic compliance, the left side of the heart begins to fail. This leads to higher diasolic filling pressures to maintain SV and CO. This **increase in pressure is transmitted backwards to the left atrium and pulmonary veins where it can cause pulmonary HTN** and subsequent R sided HF The pathogeneisis of PHTN begins with passive increase in pulmonary capillary and arterial pressure **due to pulmonary venous congestion.** This results in endothelial damage and decreased release of NO. Over time, see remodeling of the pulmonary vasculature with increased smooth muscle cell proliferation
65
4 types of hyper lipoproteinemias and what are their major side effects 1. Familial chylomirconemia 2. Familial hypercholesterolemia 3. Familial dysbetalipoproteinemia 4. Familial hypertryglyceridemia
1. can lead to acute pancreatitis 2. Can lead to premature coronary artery disease 3. Can also lead to premature coronary artery disease 4. obesity and insulin resistance
66
Vincristine/Vinblastine toxicity | (think tarzan sketch)
**Vincristine specifically: Peripheral neuropathy and neurotoxicity,** also can cause paralytic ileus and constipation also cause alopecia These drugs **bind beta tubulin and inhibit its polymerization into microtubules --\> prevent mitotic spindle formation** **\*Arrests in M phase\***
67
Highlights of the path of the median nerve
Receives contributions from **C5-T1**. Courses **with brachial artery** in groove **between biceps brachii and brachialis muscle**. Courses **between humeral and ulnar heads of pronator teres**. Then travels **BETWEEN flexor digitorum profundus and superficialis** before entering the wrist within the **flexor retinaculum**
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69
Mean +/- 1.96 SD/sqrt n vs Mean +/- 2.58 SD /sqrt n
The first one covers 95% of the population (1 standard deviation) The second one covers 99% of the population (2 standard deviations) the 1.96 and 2.58 represent Z-scores for 95% and 99% of the population respectively Standard error is SD/sqrt n and is a way to account for variability in the sample
70
Two types of hydrocephalus
Hydrocephalus is enlargement of the ventricles (with or without elevated pressure) **1. central neuronal volume loss (hydrocephalus ex-vacuo)**. Pts with neurogegenerative disease such as AIDS, dimentia can develop this due to significant cortical atrophy, which allows the ventricles to expand without increased CSF pressure **2. excessive CSF accumulation.** Typically occurs due to obstruction (noncommunicating) or absorption (communicating) 2 types below 2a. normal-pressure hydrocephalus: chronic reduction in CSF absorbtion at arachnoid villi (wet, wobby, wacky) 2b. high-pressure hydrocephalus. This is more acute, from cerebral venous sinus thrombosis, change in mental status, seizure, HA
71
Type 1 DM dx vs Gestational DM dx
Type 1 DM: fasting glucose \>126, or A1C Gestational DM: oral glucose tolerance test
72
What diabetic med for T2DM has the chance of causing hypoglycemia?
**Sulfonureas** (inhibit the K channel, cause depolarization, cause insulin release). Specifically, glyburide glipizide, glimepride Arcabose NOT associated hypoglycemia (decrease intestinal glucose absorption) Metformin NOT associated hypoglycemia (increase gluconeogenesis) Pioglitazone NOT associated hypoglycemia (increase insulin sensitivity) Staglipton NOT associated hypoglycemia (DDP-4 inhibitor)
73
Pt with HIV experiecnes fever, dyspnea, fatigue and the following stain was seen, what do they have?
**Candida albicans!** Candida fungi exist in yeast single cell form. They appear as budding yeast with pseudohypahe. The germ tube test is diagnostic of candida albicans and allows differentiation from other types of candida **These growing hyphae are called germ tubes**
74
Addition of primaquine to malaria treatment? (think warrier jumping in)
**Used to prevent disease relapse of Ovale and Vivax**, which are unique in the fact that they can establish a l**atent hepatic infection i**n the form of **hypozonites**, responsible for relapses **Chloroquine** is effective against cloroquine-sensitive plasmodium but has **NO activity against latent infection established by vivax and ovale**
75
Important time milestones after myocardial infarction 0-4 hrs 4-12 hrs 12-24 hrs 1-3 days 3-7 7-10 10-14 2weeks-2 months
76
opsoclonus-myoclonus ataxia syndrome is associated with what tumor syndrome (paraneoplastic for what)?
**"dancing eyes dancing feet"** Associated with **neuroblastoma** in children and small cell lung cancer in adults nonrhythmic conjugate eye movements This is a paraneoplastic syndrome associated with neuroblastoma. This tumor has increased copies of N-myc amplifciation
77
What organ would you see this pattern in? increased vascular resistnace with decreased oxygen content?
This would be seen in the LUNGS and is called hypoxic vasoconstriction so that blood flow is diverted away from underventilated areas. This is reverse in the rest of the system where hypoxic tissues receive increased blood flow
78
alanine and its role with nitrogen and alpha-ketoglutarate
the major AA responsible for transporting nitrogen to the liver for disposal. During the catabolism of proteins, amino groups are transferred to alpha-ketoglutarate to form glutamate. Glutamate is then processed in the liver to form urea
79
common causes of bacterial meningitis in adults, more specifically, what is the #1 cause?
strep pneumo is the most common cause of bacterial meningitis in adults of all ages
80
biggest differene between carcinoma and dysplasia?
reversibility Dysplasia refers to abnormal cell growth that is confined to the epithelium. Does not necessarily mean a precursor to cancer (if low grade) if high grade (carcinoma in-situ) is a precursor to invasive carcinoma
81
What are the three stop codons?
UAG (U Are Gone) UAA (U Are Away) UGA (U Go Away) The **codon RIGHT BEFORE** the stop codon will be the last protein added
82
Fun facts about the class I antiarrhymics 1A 1B 1C
Block sodium channel and inhibit phase 0 of the cardiac action potential (vertical line), thereby slowing conduction \*Remember that class Ia have moderate potassium channel blocking activity
83
What are the three different types of groin hernias and what are some fun facts about each? 1. Indirect 2. Direct 3. Femoral
Direct and indirect are both located above the inguinal ligament
84
Side effects of first generation antihistamines
anticholinergic (anti-muscarinic), alpha adrenergic, serotonergic Examples are chloramphenamine and diphenhydramine **Can cause accomodation and blurring of the eyes for close objects**
85
If this is found in the urine, what is elevated in the urine and why?
This is cystinuria, with the hexagonal shaped crystals in the urine. If found in 16 year old, would be indicative of cystinuria, AR disorder that does **not allow cystine, ornithiene, lysine, arginine (NO _COAL_) to be reabsorbed in the proximal renal tubules** leading to urine supersaturation with cystine **Dx: aminoaciduria.** The sodium cyanide nitroprusside test is a qualitative test used Treatment involves alkalinizing the urine with acetazolamide
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87
Hx of rheumatic fever and now has rumbling diastolic murmur heard best over 5th intercostal mid-clavicular. timing of the sound corresponds to where on plot? (Note this plot is NOT normal)
This pt has **mitral valve stenosis** (since diastolic). This plot shows an elevated pressure gradient between the left atrium and left ventricle. Hear high pitch opening snap followed by low pitched diastolic rumble best heard at the apex. **This corresponds to C in the first diagram when LA pressure is highest and the mitral valve is opening**
88
2 ways which calcium is removed from cytoplasm after contraction of muscle is complete
1. Na/Ca exchanger pumps calcium out of cell entirely 2. Calcium ATPase pump (SERCA) pumps Ca back into the sarcoplasmic reticulum
89
Bloom syndrome...and also label the figure
AR mutation in BLM gene that encodes for DNA helicase Presents with growth retardation, photosensitivity, facial anomalies, immunodeficiency (recurrent infections)
90
Chlorthalidone mechansim and side effects
This is a thiazide diuretic, works by inhibiting Na/Cl resorption in DCT Side effects are **Hyper GLUC** **hyperGlycemia** **hyperLipidemia** **hyperUricemia** **hyperCalcemia**
91
Timolol use and mechanism
**Non-selective Beta blocker** Used for open-angle glaucoma. Glaucoma is usually associated with increased IOP due to increased production or decreased draining of aqueous humor Aqueous humor is produced by the epithelial cells of the ciliary body **timolol works to decrease secretion of aqueous humor by the epithelial cells of the ciliary body** **NO pupillary or vision changes seen with timolol**
92
What are two equations for CO?
CO=SVxHR ## Footnote **CO=rate of O2 consumption/arteriovenous O2 content difference** **This second equation is determined by the Swan-Ganz catheter, by applying the fick principle**
93
What is the splenic RED pulp important for?
**1. Destroying aged and abnormal erythrocytes** (like spherocytes) and serving as an emergency store of blood cells and platelts that can be put into circulation if necessary **2. Clearance of circulating bacteria** that become lodged in the cords. Macrophages then present the captured antigens to B and T cells that reside in the splenic WHITE pulp **\*nearly 1/2 of the body's total immunoglobulins are produced by splenic B-lymphocytes. Splenic opsonizing antibody is of particular importance in clearance of encapsulated bacteria**
94
Glossopharyngeal nerve lesion (3 things)
1. loss of gag reflex 2. loss of general sensation in the upper pharynx, posterior tonsils, middle ear 3. loss of taste to posterior 1/3 tongue
95
Tongue innervation
Taste ant 2/3 --\> chorda tympani Sensation ant 2/3 --\> Trigeminal V3 Taste posteior 1/3, sensation posterior 1/3 Glossopharyngeal
96
This lesion in a 64 year old man with new onset HA and newly dx cranial mass
**Glioblastoma with necrosis and vascular proliferation**. This is the most common primary brain tumor in adults. Causes typical butterfly distribution as it crosses the midline. From astrocytes ## Footnote **\*\*Pseudopalisading necrosis (focal necrosis surrounded by tumor cells)**
97
What meds can predispose pts to manic episodes?
All SSRI (sertraline) Esp if the pt has a family hx Most pts commonly present with just depression sx and no manic sx so they are started on a SSRI and then come back a few weeks later in a manic state that was induced by the SSRI.
98
Down Syndrome baby is predisposed to what sort of abdominal problem?
**umbilical hernia** This is a defect at the linea alba that is covered in skin Associated with Down syndrome, hypothyroidism and Beckwith-Wiedemann syndrome (WT2 mutation with wilms tumor, macroglossia, organomegaly) Most are asymptomatic and resolve in first few years of life
99
What effect does an increase in estrogen on thyroid hormone?
Estogen increases the Thyroid Binding Globulin levels (which accounts for roughly 70% of thyroid binding levels with the other coming from albumin) The increase in estrogen causes an increase in TBG which causes a decrease in free T3 T4 (since more is bound up). This negatively feeds back to the pituitaty and causes an increase in TSH and increase in T3/T4 until the additional TBG is bound and free T3/T4 levels are restored. **Therefore an increase in estogen --\> increase TBG --\> increased in TOTAL T4 (bound T4 + free T4) however level of free is normal so pt remains euthyroid**
100
What goes through foramen ovale?
Trigeminal nerve mandibular branch (think O for mouth) This provides sensory innervation to the face and motor innervation to muscles of mastication
101
For our purposes, the Pyrrolidonyl arylamidase test is the same as what? (PYR)
Same as bacitracin sensitivity (essentially) ## Footnote **Group A strep is bacitracin sensitive, PYR +** **Group B strep is bacitracin resistant, PYR -** **Enterococcus is PYR sensitive but no mention of bacitracin (onlt differnece)**
102
Pretibial Myxedema, exopthalmos, periorbital edema, eye movement limitations
Seen primarily in hyperthyroidism due to Graves What happens is that there is an autoimmune response to the TSH receptor Stimulate dermal fibroblasts. Infiltration of retroorbital space by activated T-cells leading to increased cytokines (TNF-a, INF-Y) to increase fibroblast secretion of hydrophilic GAGs --\> increased osmotic swelling
103
What do the areas under the curve mean and what happens when you change cutoffs?
Basically, just know the big categories and when the line is moved left or right it either increases or decreases that exact value
104
AR mutation effect 1/4000 indivudals, what is probability that have second child affected? (think hardy-weinberg)
p+q=1 P2+2pq+q2=1 They gave us q2= disease frequency so take srt and get 1/200 and can assume other parent's contribution is essentially zero so p=0 so then you have 2pq=2 x 1/200 = 1/100 **Then in sum, the probablility of having another child that is affected is 1/4 x 1/100 = 1/400**
105
Pt c/o increased sensitivity to everyday sounds and withdraws socially as a result. What nerve/muscle/region is affected?
**Hyperacusis (increased sensitivity to certain frequencies of sound)** This is a **middle ear problem** The stapedius muscle arises from the wall of the tympanic cavity and inserts on the neck of the stapes. It is innervated by the stapedius nerve, a branch of the **facial nerve** **Note, ipsilateral hyperacusis is a common finding associated with Bells Palsy**
106
Hypertrophic or disfiguring scars can be caused by what?
Persistently **elevated transforming growth factor Beta (TGF-B)**. It is critical for stimulating connective tissue synthesis and remodeling of the extracellular matrix If TGF-B is elevated, can **lead to increased fibroblast proliferation and activity and eventual increase in collagen deposition** **=keloid scar**
107
Pure red cell aplasia
a rare form of marrow failure characterized by severe hypoplaisa of erythroid elements in the setting of normal granulopoeisis and thrombocytopoesis ## Footnote **associated with thymomas** **Can also result from Parvovirus B19**
108
Cleft Lip
**Failure of fusion of the maxillary prominence at the intermaxillary segment** to form the upper lip and the primary palate. If one of the maxillary prominences fails to fuse, bilateral cleft lip ensues
109
Person in Massachusettes comes in with fever and malaise for 48 hrs. Blood smear is shown below. What is the vector and what disease is it causing?
The vector is the **Ixodes Tick** and the disease is **Babesiosis**. The NE USA exposure and summer is indcative of Babesiosis (notice pt did not travel to Africa to obtain Malaria although images look similar) This is the **same vector for Borelli Burgdorferi (Lymes)** \*Remember that Malaria is spread by **Anopheles**\* **Aedes** spreads Chikungunya and Dengue\*
110
What hormone stimulates bile release
CCK It functions to increase pancreatic enzyme secretion (by acinar cells) AND gallbladder contraction, and to decrease gastric emptying
111
Type I Diabetic in DKA, where is the glucose being produced from? More specifically, where in the body does this process occur?
**Increased triglyceride breakdown in adipose tissue due to insulin deficiency.** TG are broken down by **hormone sensitive lipase** to glyerol and FFA. **\*Remember that adipocytes are unable to metabolize glycerol (kinda like they cant do gluconeogenesis b/c no glucose-6-phosphatase)\*** Therefore, they transport to liver where it is phosphorylated by **glycerol kinase\*\***
112
Pt presents with firm palpable abd mass as shown in image, what are they predisposed to with this?
This is porcelain gallbladder (can see the thickening walls) These pts present with RUQ pain but can be largely asymptomatic with findings discovered on PE or imagine Potential manifestation of chronic cholecystitis and is often found with multiple gallstones **These pts have an increased risk of ADENOCARCINOMA of the gallbladder** **Think of gallbladder as gland-like, so get gland like cancer**
113
Organophosphate poisoning acts where? ...and label the diagram...
Acetylchoninesterase inhibitor (**irreversibly inhibit AChE)** Need to **give them an Achetylcholinesterase**s that increase ACh (atropine, pralidoxime)
114
Panic Disorder
Recurrent, **unexpected** panic attacks. Ususally there is concern about another attack occuring. Usually have CP, SOB tachycardia, GI distress. ## Footnote **Key is that some events are spontaneous with no obvious trigger**
115
Pt has PTH independent conversion of 25-vit D to 1-25 Vit D by 1 alpha hydroxylase
This can happen with pts who have granulomatous disease (sarcoidosis, TB) due to expression of 1-alpha hydroxylase in macrophages This leads to increased intestinal calcium absorption with subsequent hypercalcemia and hypercalciuria
116
Flutamide (for rising PSA)
**Non-steroid agent that acts as a competitive testosterone receptor inhibitor** This blocks the stimulatory effect of androgens on the primary tumor and metastasis and leads to **REDUCTION IN THEIR SIZE, which improves sx such as bone pain and urinary obstruction**
117
E. Coli 0157:H7 and Shiga Toxin both work via what mechanism?
Inactivate the 60S subunit leading to an inhibition of protein synthesis and eventual cell death
118
Ureter tracks over/under what vessels?
The **ureter runs UNDER the gonadal vessels** (WATER UNDER THE BRIDGE) and then **runs OVER the internal iliac** and then **UNDER the uterine artery** **The ONLY OVER IS THE INTERNAL ILIAC**
119
What happens to the vessel in a hypertensive crisis?
**Hyperplastic arteriolosclerosis** presents as an **onion-like concentric thickening** of the walls of arterioles as a result of laminated smooth muscle cells and reduplicated basement membranes
120
How do you reduce central venous catheter infections?
**1. Hand hygeine with alcohol sanitizer or soap and water** 2. Maximal barrier precautions 3. Preparation of the sterile site with chlorhexidine 4. Use of subclavian or jugular (NOT femoral, has higher rate of infection) \*Catheter replacement at scheduled dates is NOT effective
121
Acute porphyria and what is the treatment?
An AD disorder of the heme synthesis pathway caused by **porphobilinogen deaminase deficiency.** The symptoms are the 5 P's...P**ainful abdomen, port wine- colored urine, polyneuropathy, psychological disturbances, precipitated by drugs** (cytochrome cyp450 INDUCERS), alcohol, starvation Treatment: **GLUCOSE** or heme to downregulate ALA synthase
122
Family history of colorectal cancer, endometrial cancer, ovarian cancer. What is syndrome and what are genes affected?
This patient has a family hx of **LYNCH SYNDROME!! = HNPCC** The genes affected are **MSH2, MLH1, MSH6, PMS2** Colon cancer occurs in ages \<50 due to inherited mutation in mismatch repair **Mismatch Repair = MSHhhmash**
123
sudden severe HA, bitemporal hemianopsia, decreasd libido over past few months. What is dx?
Pituitary apoplexy (problem originally was pituitary adenoma : sudden hemorrhage of pituitary gland, often in the presence of an existing pituitary adenoma.
124
What is the triad of Wernicke's, what is deficient, what is treatment, and what can make it WORSE
Triad of: **confusion, opthalmoplegia (paralysis of muscles in eye), ataxia** Due to **damage of medial dorsal nucleus of thalamus, MAMMILLARY BODIES** Thiamine deficiency is the ultimate cuase of this. IF treat with **glucose BEFORE thiamine, can make worse**. Thiamine is a cofactor for the enzymes responsible for glucose metabolism. Infusion of glucose will exacerbate pre-existing thiamine deficiency -----\> precipitating Weknicke
125
Peptostreptococcus, Fusobacterium
These are **normal flora of the mouth.** If these are found in a lung abscess, it is likely they were **aspirated**. There are several thoughts here, either the patient is an **alcoholic** and they aspirated, or they have a **seizure** disorder, or **drug overdose,** or severe **neurologic disorder**
126
What are these cells, what malignancy, and what stain are we looking at?
These are **auer rods** in myeloblast (large cells with abundant cytoplasm) These are seen in **acute myelogenous leukemia** They stain for **peroxidase**
127
Pancreatic tissue encircling (forming **RING**) the duodenum. What is it and what is causing it?
**Annular Pancreas:** ventral pancreatic bud abnormally encircles 2nd part of duodenum: forms ring of pancreatic tissue that may cause duodenal narrowing and NON-billious vomiting. This rotation behind the duodenum by the vental pancreatic bud happens during the 7th week of development Ventral pancreatic bud --\> uncinate process portion of the head and main pancreatic duct
128
What cardiac drug can redistribute blood away from ischemic areas and exacerbate myocardial ischemia
**Adenosine, _Dipyridamole_, and Regadenoson** preferentially vasodilate coronary vessels. These drugs can r**educe collateral blood flow.** Areas of ischemia are already maximally dilated. These drugs cause vasodilation in other nonischemic regions and thus leads to decreased perfusion pressure within the collateral microvessels, diverting blood from ischemic areas to non-ischemic areas and can make ischemia worse **--\> often used in myocardial imaging and perfusion studies to stimulate coronary vasodilation that occurs during exercise =Coronary steal phenomenon**
129
What artery is most likely damaged in femoral neck fracture?
Medial circumflex artery Injury to this vessel can lead to osteonecrosis of the femur head
130
What type of tumor is this pictured? Pt has newonset tonic clonic seizure. HA at night that wake pt. Pt is 62 years old male
This is a **glioblastoma** that is the most common primary cerebral neoplasm in adults. They **arise from astrocytes** and typically located in **cerebral hemispheres**. May occasionally cross the corpus callosum (**butterfly)** They also can **cause midline shift** which is seen in the third ventricle
131
Symptoms of Pellagra (what are they and what is deficient) combined with excessive amounts of neutral amino acid in the urine is called what?
Pellagra is deficiency in B3=niacin. Sx are 3D's: diarrhea, dermatitis, dementia (overdose causes facial flushing) **_Hartnup Disease_** is autosomal recessive deficncy of neutral amino acid transporters in proximal renal tubular cells and on enterocytes. Get pallagra-like symptoms and **treat with high protein diet and nicotinic acid (daily niacin)**
132
Ribavirin use (2) and mechanism
Use in **Hepatitis C therapy and RSV** (although palivizumab is preferred in children) ## Footnote **Interferes with duplication of viral genetic material**
133
Which trisomy is associated with cleft lip/palate ## Footnote **(does eddy have a cleft palate?)**
Patau, 13 NOT EDWARDS (eddy does not have a cleft lip/palate)
134
Which hepatitis are enveloped/nonenveloped
**A and E are nonenveloped** (n**A**k**E**d). Since they lack an envelope, they are not destroyed by the gut Vowels hit your Bowels (A and E, not B,C,D) The only two that are spread fecal/orally Also have **NO hepatocellular carcinoma risk**
135
Where do B-blockers act on the RAAS system?
**B-blockers INHIBIT renin release via B1 blockage on JGA cells** They have NO effect on ACE and therefore do NOT affect bradykinin levels
136
TCA overdose
**Use sodium Bicarb** Have prolonged QT and wide QRS can lead to torsades Can present with delerium, seizures Symptoms are caused by blockage of cardiac fast sodium channels **Wide QRS or ventricular arrhythmia is an indication for sodium bicarb therapy**
137
Decorticate vs Decerebrate posturing, what are their actions and where is the lesion likely
**Decorticate** (protect the CORE): **see flexion of the upper limb.** Here, the lesion is **BEFORE** the red nucleus. Due to loss of descending inhibiton of the red nucleus and subsequent hyperactivity of the upper limb flexors. **This would be damage in cerebral hemispheres, internal capsule** **Decerebrate:** see **extension of the upper limb** predominate. Here, the lesion is **AFTER** the red nucleus leading to loss of descending excitation to the upper limb flexors (via rubrospinal tract) and extensor predomiance (due to unopposed vestibulospinal tract). **This would be damage in midbrain, tegmentum, pons. This is WORSE PROGNOSIS**
138
Crossover study
Subjects randomly assigned to one of two groups. Take med first then placebo or placebo first and then med. Essentially, the subjects act as their own control
139
Immigrant who did not get vaccinated gets rash from face that spreads to body, what do they have and if they are pregnant what will they have and what will the kiddo have?
They did not receive their MMR vaccine. They have Rubella (german measles). Rash starts on face and spreads to extremities. **Mom --\> polyarthralgia** **Infant --\> sensorineural deafness, cataracts, PDA**
140
Circulating IgG4 antibodies to phospholipase A2 (PLA2R) receptor is indicative of what?
**Membranous Nephropathy:** remember this is the most common cause of primary NEPHROTIC (can be nephritic too though) syndrome in Caucasian males. **See spike and dome appearance with subepithelial deposits** This is caused by HBV, HCV, solid tumors, SLE, NSAIDS penicillamine (Wilson)
141
What does malonyl-CoA do?
-First, it is a **substrate in the synthesis of fatty acids** (palmitate, specifically) Since it is involved in the SYNTHESIS of fatty acids, it makes sense that it also **INHIBITS the enyme responsible for breakdown of Fatty-Acyl CoA (which is carnitine acetyltransferase)** Do not want to break down the FA that you JUST synthesized
142
Pt cannot sleep, what are some examples of stimulus control therapy?
**Use bed only for sleep and sexual activity** **Go to bed only when sleepy** **Leave bed when unable to sleep and go to another room** **Fixed wake-up time, even on weekends** --\>these are different from sleep hygeine which includes regular sleep schedule, no caffeine after lunch, no alcohol or meals near bedtime, do NOT exercise right before bedtime
143
What viruses bud through and acquire their envelope from the host cell NUCLEAR membrane
The herpesviruses do this (including cytomegalovirus, EBV, HSV, VZV, HHV8...)
144
Pt presents LIKE superior vena cava syndrome but is UNILATERAL sx on right only
Right sided **brachiocephalic vein obstruction**. May be the result of external compression of apical lung tumor or thrombotic occlusion as can occur when a central catheter has been placed for a significant amount of time
145
RIGHT homonomous hemianopia
This would be do to **contralateral optic tract lesion** Remember the optic nerve goes back from the eye itself and then crosses at the optic chiasm and then turns into the optic TRACT
146
Narcolepsy cause
Caused by **decreased hypocretin (orexin)** production in the lateral hypothalamus. This is **detected in the CSF** **Cataplexy** is loss of all muscle tone following strong emotional stimulus such as laughter or joking \*\*Note that the level of melatonin is NOT decreased in these pts. The level of melatonin IS decreased in Alzheimer pts
147
Low PaCO2 in setting of acute onset dyspnea, hypoxemia and hypocapnia is due to what?
Alveolar hyperventilation. PaCO2 is inversely related to and considered to be the main indicator of total alveolar ventilation **hypocapnia --\> alveolar hyperventilation** Can result from a V/Q mismatch resulting in decreased CO2 and O2 exchange leading to hypoxemia stimulating resp drive centers above normal levels. This leads to excessive CO2 excretion resulting in hypocapnia
148
Pt presents with asymetric smile, effacement of right nasolabial fold and mouth drawn towards left side. What condition does this pt most likely have and what are the common findings and what else would we expect to see on her?
This pt has a **Bells Palsy (Facial nerve palsy)** These pts present with sudden onset unilateral facial paralysis with **mouth being drawn to UNAFFECTED** SIDE. Pts also have **DECREASED tearing**, and **hyperacusis** (increased sensitivity to certain frequency sounds)
149
COPD pt 88% O2 on room air using accessory muscles with expiratory wheezes is started on high flow O2 and becomes increasingly lethargic and confused. WHY??
**Oxygen induced hypercapnia** in COPD In a COPD exacerbation, these pts need O2, but NOT high levels of O2. High oxygen concentrations can lead to increased CO2 retention resulting in confusion and lethargy. There are three mechanisms resonsible for this phenomenon. 1. The first is that you are chronically pulmonary vasoconstricted with hypoxia and now this is reversed, and you increase physiologic dead space. 2. The second is due to the high affinity of CO2 for Hb. With high O2, get Haldane effect where you decrease Hb affinity for CO2 and it gets dumped into plasma. 3. The third is that the chemoreceptors sense O2 and then decrease the need for ventilation, so the CO2 builds up
150
Infusing NE and it infiltrates into skin around IV. The skin is white, cold, and hard. What do you give?
**NE is a potent alpha-1 agonist** (**a1\>a2\>b1, NO b2)** leading to potent vasoconstriction, which can lead to local tissue necrosis Thus, it would be logical to given alpha-1 antagonist. **Phentolamine** is a great example, as this would block the alpha receptor **\*\*Remember that phentolamine is the same med that you give to pts on MAO-I who eat tyramine-containing foods\*\***
151
What TB drug causes orange fluids to come out and what is the mechanism of this drug?
This is **Rifampin** The mechanism is it **binds bacterial DNA dependent RNA polymerase** thereby preventing transcription and leading to a deficiency of proteins necessary for TB survival
152
Pt with increased ICP and potential head injury is treated with specific med. A few hours later, shows evidence of pulmonary edema. Wat med and WHY?
This pt was given mannitol, an osmotic diuretic used in the management of cerebral edema and increased ICP. It works by increasing plasma osmolality, which causes fluid to move from the intravascular space into the vessels. Overagressive treatment can lead to excess volume depletion and eventual hypernatremia Additionally, this rapid increase in vascular volume can lead to PULMONARY EDEMA
153
3 day old infant with bilious vomiting. See compression of duodenum by fibrous bands extending from the cecum and right colon to the retroperitoneum. What does this baby have and why?? Moreover, what process failed to happen?
This baby has **Volvulus,** which is **twisting of a portion of the bowel around its mesentery.** This **can lead to obstruction and infarction.** Initially get 90 degree couterclockwise rotation outside of gut. --\> stays outside and grows for 8-10 weeks --\> returns and rotates another 180 degreees counterclockwise (**for total of 270**). Incomplete rotation will result in midgut malrotation. The cecum will rest in the RUQ and the fibrous bands that connect the RUQ to the retroperitoneum (Ladd's bands) will pass over the duodenum and compress it. **Additionally, the mesentary is vulnerable to twisting around the superior mesenteric artery**
154
Beck's Triad and what is it for? | (cardiac)
**1. Pulsus paradoxicus** **2. Distended neck veins** **3. Hypotension** This is due to cardiac tamponade (compression of the heart by fluid)
155
156
The thymus is derived from what embryologically? What else is derived from this? And what disease is commonly associated with thymoma or thymic hyperplasia?
It is derived from the **third pharyngeal pouch**. This is the same as the **inferior parathyroid glands.** **Myasthinia Gravis** is often associated with thymomas
157
Selective arterial vasodilators. What are a few examples and what sort of schenanigans can we expect from them?
Hydralizine and Minoxidil are two examples They lower blood pressure by reducing systemic vascular resistance (via their arterial vasodilaiton). This effect is limited by subsequent stimulation of baroreceptors leading to sympathetic activation. This leads to increased HR, contractility, and CO. This ALSO increases activation of RAAS system, which would increase Na and water reabsorption For this reason, they are rarely used in management of HTN, but can be used acutely for malignant HTN (esp if given with diruetics and sympatholytics)
158
159
What is the interaction between beta blockers and hypoglycemia. And which kind of beta blockers do this?
nonselective beta blockers inhibit NE/Epi mediated compensatory reactions to hypoglycemia. Blocking B2 also inhibits gluconeogenesis and peripheral glygogenolysis Nonselective beta blockers should be used in caution in pts with diabetes as these can increase the risk of hypoglycemia and reduce patients awareness of hypoglycemia **Selective B1 bblockers are preferred (A--\>M)**
160
Treatment of Toxoplasma Gondii (would show up as multiple ring enhancing lesions on brain CT) (Think rabbits and cat poop)
Treat with **Sulfadiazene and Pyrimethamine along with Leucovorin (folinic acid)**
161
Selegiline (2 uses)
Inhibitor of MAO, used in parkinsons disease to delay the progression Can also be used to treat MPTP (street drug) since the active metabolite MPP+ once broken down by MAO is what is actually causing the damage
162
What aspect of collagen synthesis occurs extracellular
N-terminal peptide removal by N and C procollagen peptidases occurs OUTSIDE of cells Collagen molecules then spontaneously assemble Covalent cross links are then formed by lysyl oxidase
163
Where does nyacin come from? (2)
1. Diet (deficient in populations who eat primarily corn, alcoholics, chronic illness) 2. Synthesized endogenously from **tryptophan** It is a essential component of the enzymes NAD and NADP, which participate in redox metabolism
164
In cirrhosis what vein is backed up into to create esophageal varicies
Left Gastric
165
Pt has MS, what CONSTANT (length, time, space) is decreased due to destruction of myelin
**Length constant:** measure of how far along an axon a signal can propagate. A low length constant reduces the distance an impulse can travel. Myelin increases the length constant and decreases the time constant (improving axonal speed) \*Note, the time constant is the time it takes to get from one spot to another spot. This number is INCREASED in MS since the signal takes a longer time to get from A to B
166
Prolonged administration of glucocorticoids as seen in a pt with SLE
**Iatrogenic Cushing Syndrome** Can suppress the entire HPA axis leading to low levels of ACTH cortisol. Prolonged suppression of ACTH can lead to **bilateral atrophy of adrenal cortices** Sudden cessation of glucocorticoids can cause adrenocortical insufficiency and adrenal crisis (reason why need to taper!)
167
Causes of Myopathy with increased CK (3) and (2) with normal CK
**_ELEVATED CK_** 1. Statin Induced 2. Inflammatory myopathy (dermatomyositis, polymyositis) 3. Hypothyroid myopathy (muscle pain and cramps and weakness involving proximal muscles **_NORMAL CK_** 1. Glucocorticoid induced myopathy 2. Polymyalgia Rheumatica
168
First, second, and third line treatment for acute gout flair
1. NSAID 2. Colchisine 3. Glucocorticoids (only if cannot tolerate first two). Esp contraindicated if pt has DM since it will increase his glucose levels
169
Pt has Cushing Syndrome and cortisol is suppressed by high dose (but not low) dexamethasone. Pts ACTH levels are increased.
Since the ACTH levels are increased, it is either pituitary adenoma or ectopic producer. Since the cortisol was suppressed with the Dex test, we know that it cannot be ectopic because it actually responded to treatment. Thus, we know it must be a **pituitary adenoma**
170
What common drug metabolizes 6-mercaptopurine
**Xanthine oxidase _INACTIVATES_ it** **HGPRT activates the drug** This effect is so significant that chemo pts must reduce their dose of 6-MP by 70% if they are taking allopurinol. This is because the coadministration can lead to high levels of 6-MP metabolites **This drug is a purine analog that decreases de novo purine synthesis from PRPP --\> IMP, so there would be decreased AMP and GMP. It is used in preventing organ rejection, RA, IBD, SLE, and ALL. Side effects are myelosuppression, hepatotoxicity**
171
What is seen on this image below? (infant)
This kiddo has **necrotising enterocolitis**, more specifically he has pneumatosis intestinalis (air in the small bowel). In an infant, these findings on X-ray would be consistent with necrotising enterocolitis It affects predominantly preterm infants secondary to GI and immunologic immaturity. Impaired mucosal barrier allows the bacteria to invade the intestinal wall causing inflammation and ischemic necrosis. **Becomes gangrenous and gas develps** 30% of neonates die, esp when disease is complicated by intestinal perforation
172
Immigrant from Peru presents with difficulty swallowing liquids. Absent peristalsis in smooth muscle portion of esophagus. What bug?
This pt has **Trypanosoma Cruzi, which can cause Chagas Disease (caused by Reduviid, kissing bug)** It produces a neurotoxin that destroys the myenteric plexus and causes intramural parasympathetic enervation of smooth muscle
173
Dystonia
Neurological movement disorder characterized by sustained, involuntary muscle contractions
174
Glucagonoma
Tumor of **pancreatic alpha cells** --\> overproduction of glucagon Sx: **dermatitis (necrolytic migratory erythema), DM, Diarrhea, declinging weight, depression** **ALL D's** Dx: look at glucagon levels
175
Tumor has EGFR mutation, treat with anti-EGFR but additional mutation makes this ineffective. What is this mutation?
KRAS mutation making the anti-EGFR ineffective. KRAS is a proto-oncogene that encodes a GTP binding protein involved in regulating cell division via transduction of extracellular signals such as those from EGFR KRAS mutations are found in a lot of metastatic colon cancers
176
What froms the fibrous cap in atherosclerosis
**Smooth muscle cells** The infiltrating macrophages and T-lymphocytes release cytokines (PDGF, fibroblast growth factor, endothelin-1, interleukin-1) that attract migration and promotion of vascular smooth muscle cells (VSMC) within the intima. These are stimulated and synthesize extracellular matrix proteins (collagen) that form the fibrous cap typical of mature atheromas
177
recurrent pyelonephritis and HTN in a 12 year old girl with recurrent fever and abd pain caused by what?
Vesicoureteral reflex (VUR) Pts with this are at much higher risk of chronic pyelonephritis. Ongoing injury leads to renal scarring most commonly at the upper and lower poles. If untreated, can lead to HTN
178
Label the figure. Also, 4 year year old boy with HA and nonbilious emesis 2 weeks occuring early morning. Blurry vision when looking up with bilateral papilledema, inability to gaze upward. Where is the lesion
This patient has a **pineal gland mass = Pinealoma** Clincally, they present with **Parinaud syndrome** (compression of tectum --\> vertical gaze palsy: only want to look down) **See B-hCG production** Most common pineal gland mass is a **germinoma**, a malignant midline tumor that is thought to develop from embryonic germ cells A. Pituitary B. Thalamus C. Pineal Gland
179
Why is DNA polymerase **1** unique?
It has **5'--\>3' exonuclease activity** (removes RNA primer and repair damaged DNA)
180
Broad based budding of yeast Pseudohyphae with blastoconida Right angle branching hyphae
Broad based budding --\> Blastomyces Pseudohyphae with blasoconida --\> Candida (central venous catheter with TPN is a high risk factor for this) Right angle --\> Rhizopus species
181
Coronary dominance is determined by what? And where is this artery derived from in different dominant groups? What is the relationship to the AV node with this?
coronary artery that provides blood to the **posterior descending artery** (posterior interventricular artery) The PDA originates from one of the following: 1. Right coronary artery (70% people, Right heart dominant) 2. Left circumflex artery (10% of people, Left heart dominant) 3. Both left and right circumflex in 20% of population (codominant) **\*Note, the AV node blood supply usually is derived from the dominant coronary artery\***
182
Conversion disorder
neurologic symptoms incompatable with any known neurologic disease. Often acute onset associated with stress (pt breaks up with boyfriend and then has strange leg pain with all tests coming up negative. This patient DISLIKES doctors and does not want to be there, which differentiates it from factitious disorder where the patient WANTS to be the sick patient)
183
What nerve innervates the quadriceps muscle and where is a good place to block this nerve?
**Femoral nerve, block using femoral nerve block** and the best place to do so is the **inguinal crease** at the lateral border of the femoral artery NOT in the femoral ring, the upper opening of the femoral canal (too high)
184
Attributable risk percent in the exposed?
ARPexposed=100 x ((risk in exposed-risk in unexposed)/risk in exposed)) ARP exposed =100 x ((RR-1)/RR) so if RR was 5, it would be =100 x (5-1)/5) = 80%
185
CD31, what is it on and what does it indicate from a cancer perspective?
PECAM1 (platelet endothelial adhesion molecule), this indicates the tumor has arisen from vascular endothelial cells
186
Some fun facts about a craniopharyngioma
Most common childhood tumor can cause bitemporal hemianopia. Derived from remnants of Rathke's Pouch --\> anterior pituitary Calcification is common, Cholesterol crysals found in "motor oil"- like fluid within tumor.
187
Long term Omeprazole therapy is associated with what?
Increased risk for osteoperosis
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Half life equation Maintenance dose equation Loading dose equation \*\*\*\*KNOW THEM ALL\*\*\*\*\*
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CRACK AMIGOS in GQ inhibit success
Cimetidine Ritonavir Amiodarone Ciprofloxacin Ketoconazole Acute Alcohol Abuse Macrolides Isoniazid Grapefruit juice Omeprazole Sulfonamides Gemfibrozil Quinidine
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Bull S\*\*t CRAP GPS induces chronic madness
Barbituates St. John Wort Carbamezapine Rifampin Alcohol chronic (chronically induce cirrhoris) Phenytoin Griseofulvin Phenobarbitol Sulfonurea
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Pt with latent TB on INH comes in with severe fatigue and microcytic anemia
Pt has **sideroblastic anemia from INH** **Decreased activity of ALA synthase** INH directly **inhibits pyridoxine phosphokinase**, which normally convers pyridoxine (vit B6) to its active form), which is a cofactor with ALA synthase in the heme synthesis pathway
192
Heritable pulmonary hypertension
often due to inactivating mutation **in BMPR2** (normally **inhibits vascular smooth muscle proliferaiton.** Transmitted AD with variable penetrance
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Label this figure, and what is the mnemonic, and what is happening in the dotted line
The difference between B and C depicts the volume of blood ejected With the dotted lines, this patient has increased contractility with a larger volume of the blood ejected. as a reult there will be an increase in stroke volume or ejection fraction
194
What can you give a measles (rubeola) kiddo because they are probably deficient in it? (vitamin)
**Give them Vitamin A.** (think measles/weasles with vitamin A hats) Acute measles infection depletes Vitamin A stores and giving supplemental Vitamin A. Vitamin A helps prevent and treat these ocular complicaitons. It reduces length of hospital time and recovery time NO OTHER VITAMIN IS LINKED TO MEASLES
195
What is the nontraditional way Down Syndrome is inherited?
Translocation Down Syndrome --\> can be inherited from a noninfected parent with a balanced translocation This robertsonian translocation occurs between chromosomes 21 and 14. The result is a fusion of 2 long arms and fusion of two short arms (nonessential genetic material) The resulting fetus has an unbalanced robertsonian translocation with 46 chromosomes and 3 effective copies of 21 (46 XX, t(14;21))
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Osgood-Schlatter Disease
overuse injury of the second ossification center (apophysis) of the tibial tubercle VERY COMMON knee pain in young adults after a recent growth spurt **\*\*Pain and swelling at the tibial tubercle, the intsertion point of the patellar ligament\*\*** Repetitive contraction of the quadriceps (jumping) results in avulsion fractures causing the proximal patellar tendon to separate from the tibial tubercle
197
What aspect of Korsikoff Werkicke is perminent?
The **memory loss and impairment of learning is usually perminent** The ataxia, confusion, occulomotor dysfunction are all reversible with **THIAMINE +Glucose** **\*\*\* Remember, thiamine must be given at same time or before glucose, or will worsen the whole shebang\*\***
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Arginase
The enzyme responsible for converting arginene into urea and ornithiene at the end of the uric acid cycle. If deficienct, there would be a buildup of arginine (seen in blood) and can present as bilateral leg stiffness and abnormal involuntary movements, and growth delay This pt will NOT present with hyperammonemia Treatment includes arginine free, low protein diet
199
Hawthorne Effect vs Pygmalian Effect vs Berkson bias
**Hawthorne effect:** The tendency for subject studies to change their behavior because they know they are being studied **Pygmalian effect:** this is the tendency that physicians/researchers's beliefs in the efficacy of a treatment can potentially affect the outcome **Berkson Bias:** refers to a selection bias chosing hospital patients as a control group (less healthy than general population, will skew results)
200
Pt has stable angina and needs secondary prevention but has an allergy to ASA, what can you use instead
**Clopidogrel**: reversibly blocks P2Y12 component of ADP receptor on the surface of platelet Clopidogrel is as effective as ASA for prevention of CVA events The pt is unable to tolerate ASA due to exacerbation of preexisting respiratory sx
201
Postauricular and occipital lymphadenopathy more common in Rubella, Rubeola? ## Footnote **Germans get neck swelling**
This is a characteristic of RUBELLA (German Measles), a togavirus ## Footnote **Germans get neck swelling**
202
Cluster of enlarged tortuous veins in the gastric fundus (ONLY). Increased pressure in what vessel is causing this. Pt has history of chronic pancreatitis
Gastric varicies can be seen in splenic vein thrombosis due to chronic pancreatitis, pancreatic cancer and abdominal tumors The **SHORT GASTRIC VEIN** drains the fundus into the **splenic vein**
203
what enzyme in the free radical pathway NEUTRALIZES the free radicals?
**Superoxide dismutase** converts superoxide to hydrogen peroxide Note: NADPH oxidase converts O2 to O2- (superoxide), and myeloperoxidase converts H2O2 to HClO (
204
Metabolism yields how many calories per gram: Carb Fat Protein Ethanol
Carb: 4 Fat: 9 Protein: 4 ETOH: 7
205
This pts forehead has what? and what is the biggest concern?
This patient has **Actinic Keratosis:** small scaly erythematous lesions with a sandpaper texture occuring on sun exposed areas AKs do NOT invade the dermis and are considered to be premalignant lesions --\> **invasive squamous cell carcinoma**
206
Small bluish lesion underneath nail of right index finger. If cancerous, what are the cells of origin and what is the function of these cells?
May be a **Glomus Tumor or a Subungal Melanoma** In this example, Gloumus is more likely choice since no hyperpigmentation was seen This is a tumor of modified smooth muscle cells of a glomus body. A glomus body is part of the dermis layer of the skin involved in body temperature regulation. Each glomus body is attached to an arteriovenous anastamosis. The role is to shunt blood away from skin in cold temperatures and direct blood to surface in hot temperatures to facilitate the disipation of heat **TEMPERATURE REGULATION**
207
Moderately elevated ALP should be followed by what test?
**gamma-glutamyl transpeptidase (GGTP)** This is increased in various liver and biliary diseases (just as ALP) but NOT in bone **\*\*Good way to distinuish if the elevated ALP is from bone origin or liver origin\*\***
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If CNS tumor stained positive for synaptophysin, what type of cell did it originate from?
Neuronal cell It is a transmembrane glycoprotein found in the presynaptic vesicles of neurons, neuroectodermal, and neuroendocrine cells
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What is going on in this picture?
This is a **chronic AV fistula**. Increased CO (increase in height of cardiac function curve), and decreased TPR (increased slope of both) and an increase in mean systemic pressure (right shift of venous curve along x-axis)
210
Glycogenolysis is the major source of fuel for ___ hours? Then what takes over
Glycogenolysis for first 12-18 hours of fasting Then gluconeogenesis takes over The first committed step of gluconeogenesis is the conversion of pyruvate to oxaloacetate by pyrvuvate carboxylase
211
Fam hz of mild hypercalcemia, and hypocalciuria with normal to increased PTH level
**Familial Hypocalciuric Hypercalcemia.** This is an AD condition where there is a defect in the calcium sensing receptors in multiple tissues (CaSR). This requires higher than normal levels of calcium to suppress PTH These are transmembrane G-protein receptors that help to regulate secretion of PTH in response to changing serum calcium levels
212
Greatest risk of dying with Fredreich Ataxia
hypertrophic cardiomyopathy
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see double when walking down stairs and when trying to read documents or newspaper. Where is the lesion?
This is a trochlear nerve palsy. This innervates the superor oblique muscle, which causes the eye to intort and depress while adducted
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Transthyretin
A protein tetramer produced in the liver that acts as a carrier of thyroxine and retinol Mutations in the gene cause a misfolded protein that results in an amyloid protein that infiltrates the myocardium (infiltrative cardiomyopathy)
215
Black vs Brown stones in gallbladder
Black: chronic hemolysis (sickle cell, spherocytosis), increased enterohepatic recycling of bilirubin (ileal disease) Brown: E. Coli, liver fluke (chlonorchis sensilis)
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Epi by itself ___ insulin levels Epi + drug A increase insulin levels, what receptor is drug A acting on?
Epi by itself decreases insulin secretion Stim of B2 promotes insulin exretion Stim of A2 inhibits insulin secretion Since epi acts on both of these receptors, it would have to be an A2 blocker if epi was now all of a sudden increasing insulin secretion
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MEN2(A and B), and what are some fun facts about the M\*\*\*\* cancer histologically
MEN2A Medullary thyroid cancer, Pheochromocytoma, parathyroid hyperplasia MEN2B: Medullary thyroid cancer, pheochromocytoma and mucosal neuromas Medullary thyroid cancer is a neuroendocrine tumor that arises from parafollicular Calcitonin secreting C-cells. These show up as nests of polygonal cells with Congo-red positive deposits
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Migratory thrombophlebitis
This should raise suspicion for visceral cancer (adenocarcinomas of the pancreas, colon, lung)
219
The first area of the brain damaged by global cerebral ischemia (like during cardiac arrest)
Hippocampus is the first area damaged (also the purkinje cells of the cerebellum)
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What is responsible for this small dip after the alveolar capillary bed?
Mixture with deoxygenated blood Blood in the left atrium has a slightly lower O2 content than blood in the capillaries. This is due to mixing of deoxygenated blood with oxygenated blood from the pulmonary veins. \*This deoxygenated blood comes from: 1. bronchial arteries carry blood to the bronchi and bronchioles (most of this blood returns via the pulmonary vein) 2. small cardiac (thesbian veins) that drain into the LA and ventricle and contribute to normal anatomic shunting
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Cholestyramine
A bile acid binding resin that binds bile acid in the GI tract thereby interfering with the enterohepatic circulation of bile acid and therefore cause increased bile acid excretion. This results in synthesis of new bile acid, a process that consumes liver cholesterol stores
223
what does RT-PCR detect?
**used to detect and quantify levels of mRNA in sample** Uses reverse transcription to create complementary DNA template that is then amplified using the normal PCR mechanism
224
Valproate is a teratogen and causes what?
Neural tube defects (myelomeningocele, meningocele)
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VIPoma
non alpha, non beta islet cell pancreatic tumor that secretes VIP This results in Watery Diarrhea, Hypokalemia, Achlorhydria (WDHA syndrome) This can be treated with somatostatin (octreotide), which inhibits VIP production
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New onset odynophagia in the setting of GERD
Usually indicates the presence of erosive esophagitis and the formation of an ulcer
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3 year old with intellectual disability dies of seizures. Autopsy shows pallor of substantia nigra, locus ceruleus, and vagal nucleus dorsalis. What condition and what enzyme?
This pt has **Phenylketonuria**, and a deficiency in **phenylalanine hydroxylase.** This enzyme requires cofactor with **BH4.** Excess phenylalanine is what causes **neurological damage** in these pts See **hypopigmentation** due to decreased synthesis of melanin The **musty body odor** is due to accumulation of phenylalanine
229
During active skeletal muscle contraction, what is the substance most likely for increasing the rate of glycogenolysis during active contraction
**Calcium** Glycogen in the skeletal muscles is used to provide energy for muscle contraction Increased Calcium is a powerful activator of muscle phosphorylase kinase When Ca is released from the SR, there is synchronyous muscle contraction (via binding troponin...) as well as glycogen breakdown via activation of PK to increase glygogenolysis
230
Endocardical cushion defect. What is it and what kiddos is it seen in?
When there is failure of endocardial fusion, this results in: **1. osteum primum atrial septal defect** **2. ventricular septal defect** **3. single AV valve** This results in significant L --\> R shunting and AV valve regurg leading to excess pulm blood flow and sx of heart failure This is commonly seen in **DOWN SYNDROME**
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FDA will approve new drug if this drug plus current tx reduce incidence by 40% compared to std alone. Std alone is 8%. What is the maximum incidence of recurrent disease acceptable if FDA is going to approve?
The new drug will be approved if its associated recurrence rate is decreased by at least 40% compared to recurrence rate on std therapy alone, which is 8%. so 40% of 8% is 0.4 x 8% =3.2%. The maximal acceptance rate would be 8%-3.2% =4.8% Basically, you are looking for a Relative Risk Reduction (RRR) RRR=(ARcontrol-ARtreatment)/AR control RRR=0.4 and ARcontrol is 0.08
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Diplopia, Dysphagia, Dystonia
Classic triad for **botulinism poisoning** (cleave SNARE in presynaptic terminal leading to flaccid paralysis)
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Pt in normal sinus rhythm, what and where is the pacemaker of the heart?
The SA node is located near where the superior vena cava enters the heart in the RA (junction of RA and SVC)
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Before starting on Dapsone, what must you check?
G6PD test because see hemolysis if give and dont check \*\*If have G6PD, could also see anemia if give: antimalaria drugs, TMP-SMX, DKA, Ingestion of fava beans
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Does Cushing syndrome cause hyperplasia or hypertrophy of adrenal (and what part of adrenal is this caused)?
Individuals with signs and symptoms of cortisol excess have Cushing Syndrome Cushing DISEASE is a type of cushing syndrome that is caused by excess ACTH from pituitary adenoma. Pts with Cushing disease usually develop **HYPERPLASIA** of the zona **fasiculata** and zona **retiularis**
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Neurokinin 1 receptor antagonist
These drugs are used as antiemetics The are postrema in the 4th ventricle has a chemoreceptor trigger zone that can respond to drugs, toxins, NT The nucleus tractus solitaris receives signals from the area postrema to cordinate the vom process **NK1 receptor antagonists prevent substance P release (aprepitant, fosaprepitant)**
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High potassium High creatinine Vision changes AFIB tx Lethargy and confusion Junctional escape rhythm w/ HR 48 with PVC WHAT MED DID THIS???
Digoxin toicity --\> cardiac arrhythmias, nonspecific GI sx, neurological and visual sx (seeing yellow) as well as hyperkalemia (via inhibiton of Na/K ATPase
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Buspirone
Stimulates 5HT-1a receptors Used in Generalized Anxiety Disorder **\*\*Takes 1-2 weeks to take effect so NO ACUTE USE\*\***
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McCune Albright Syndrome triad presentation
1. fibrous dysplasia 2. precocious puberty 3. Cafe-au-lait spots This is a somatic mutation in GNAS gene encoding the stimulatory alpha-subunit of G-protein, get constituitive activation of AC
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Thoracic Outlet Syndrome
Compression of LOWER trunk of brachial plexus as it passes through the thoracic outlet, presents as upper extremity numbness, tingling, weakness ALSO see compression of subclavian vein can cause upper extremity swelling OR compression of subclavian artery causing exertional arm pain This usually occurs in the SCALENE TRIANGLE...can be due to cervical rib, scalene muscle anomalies, injury (repetitive overhead use)
241
Pt started on TMP-SMX and develops dark urine and amenia. How explain these findings?
This pt has G6PD and this is not an uncommon way for this to present. This is hemolysis induced by the TMP-SMX G6PD is an X-linked disorder that affects mostly males and is more common in pts of African, Asian, and Med descent. it is an enzyme of the Pentose pathway and eventually results in decreased NADPH and inability to maintain glutathione in reduced state
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Most common place for atherosclerosis?
1. Abdominal artery 2. Coronary arteries 3. popliteal 4. Internal carotid 5. Circle of willis
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Sudden onset HA, 160/90 BP. Caused by what?
This is a rupture of saccular aneurysm Clinically the acute onset of HA is characteristic of subarachnoid hemorrhage and the CT confirms the dx. Focal neuro deficits are usually NOT seen Berry aneurysms are the MOST common cause of SAH with the anterior communicating artery being the most common site
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Charcot-Bouchard Aneurysms vs Berry Aneurysms
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incidence
of new cases/total population at risk
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What is a neurophysin
Carrier protein for oxytocin and vasopressin (ADH). Neurophysin bind and act like chaperone protein
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3 week hx of painless neck swelling and weakness with this biopsy. What does he have?
This is a classic Reed Sternberg cell (Owl Eye) of Hodgkins Lymphoma. These cells are derived from germinal center B-lymphocytes and are the neoplastic cells of Hodgkins \*\*They are necessary for dx\*\*
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Methylmalonic Acidemia
This is an AR acidemia from parial or complete deficiency of methylmalonyl-CoA mutase Leads to **buildup of methylmalonic acid** leading to metabolic acidosis. Hypoglycemia results from overall increased metabolic activity leading to increased glucose utilization and direct toxic inhibition of gluconeogenesis --\> **KETOGENESIS**. The organic acids also inhibit urea cycle leading to **HYPERAMMONIA**
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Achlorhydria predisposes to what?
First, achlorhydria is decreased gastric acid production to maintain normal gastric pH \<4 Predisposed to things like Cholera infection at lower innoculation dose. Normally, killed by stomach acid, but if have less acid you need less bacteria to infect. Could be low due to chronic omeprazole therapy or a result of gastritis
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Pt presents with sx of some clotting factor disorder (factor VIII, IX...), what can you give them if they are actively bleeding to stop bleeding
Thrombin will make up for the deficiency and lead to blood clotting \*Remember that in hemophilia, the bleeding time and platelet count are normal (those would be low in things like VWF, Glanzmann..). Also the PT would be normal since it really just tests Factor VII.
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Depersonalization/Derealization Disorder vs Dissociative Amnesia vs Dissociative Identity Disorder
**Depersonalization** --\> experiencing surround as unreal. Feelings of detachment **Dissociative amnesia --**\> inability to recall important personal information **Dissociative identity** --\> associated with severe trauma/abuse
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Pt is put on Atenolol. What happens to JGA cell cAMP Cardiomyocyte cAMP Vascular smooth muscle cAMP
JGA --\> DECREASE Cardiomyocyte --\> DECREASE Vascular smooth muscle --\> NO CHANGE \*\*\*Remember that Atenolol is a SELECTIVE B1 blocker that acts on cardiac myocytes and JGA cells. There are NO B1 on vascular smooth muscle cells
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Pt itches all over body (along with fatigue hepatomegaly, elevated alk phos). What is this called and what are some causes? And what happens when prolonged?
Cholestasis The causes can be intrahepatic or extrahepatic When it is prolonged, the reduction in bile flow results in malabsorption of fats and fat soluble vitamins (ADEK), which REQUIRE bile salts for digestion
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Pt has Crohn disease and terminal ilium resection, what are reprecussions?
This pt will not be able to reabsorb BILE ACIDS leading to an inability to absorb ADEK vitamins. Also inability to reabsorb B12 leading to megaloblastic anemia The K deficiency will result in decreased Factor 2,7,9,10,C,S which will lead to easy bruising and large hematomas
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Amniotic Fluid embolism
This can happen through sites of uterine trauma or cervical lacerations. Fetal squamous cells and mucin are seen on autopsy. When this arrives at the lung, it causes an anaphalactoid reaction causing occlusion and vasospasm of the maternal pulmonary circulation and can lead to DIC. Tissue factor (thromboplastin) is also released from amniotic fluid
256
What is point of adding Clavulonic Acid to Amoxicillin?
Clavulonic Acid is a beta-lactamase inhibitor that inhibits beta lactamase that was made by the bacteria in an attempt to thward the antibiotics. Other beta-lactamase inhibitors are sulbactam and tazobactam It expands the spectrum of action to bacteria that are beta-lactamase synthesizing
257
3 year old boy with neck stiffness and petechial rash all over trunk with labs that look like he is in DIC. Child dies, what caused this and what see on autopsy?
This pt has Neisseria Meningititis (know because of the neck stiffness with concurrent petechial rash all over the trunk and DIC presentation. This is highly associated with Waterhouse-Fredreichson syndrome which is bilateral adrenal hemorrhage
258
What is the cause of angioedema after taking ACE-I?
It can affect any tissue but is classically associated with lips, tongue, eyelids. Laryngeal edema and difficulty breathing may also occur When taking an ACE-I, bradykinin is accumulated. NORMALLY, ACE is responsible for bradykinin breakdown. Bradykinin is a potent vasodilator, causing significant angioedema
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Two renal drugs that prevent unfavorable cardiac remodeling in CHF
1. ACE-I 2. Spironolactone, Eplerenone (3. Beta Blockers) \*Both block deleterous effects of Aldosterone on the heart
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Triad of Henoch-Schonlein Purpura and what type of immune complex deposition seen?
1. Palpable Purpura 2. colicky abd pain 3. polyarthralgia IgA and C3 deposition seen
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Kallmann Syndrome
Defective migration of GnRH secreting neurons from their origin in the olfactory placode to their location in the hypothalamus Most often due to mutation in KAL-1 gene or FGFR-1 gene Classically have central hypogonadism and anosmia. Most often these present with delayed puberty
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Pt has been vomitting and diarrhea for past 24 hrs. BP is 90/50, what is going on with RPF, GFR, FF
**RPF: Decreased** due to a decline in circulating volume leading to a systemic vasoconstriction, including renal vasoconstriction **GFR: Reduced** RBF causes perfusion pressure to drop, this the GFR drops as well. This decreases distal tubule Na delivery, thus we see an **increase in RENIN --\> RAAS** **FF:** Remember this is a ratio of GFR/RPF. The RPF decreased more than the GFR, thus, this the FF **INCREASED**
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Pt gets colonoscopy and has small mucosal lesion. The pathogenesis of this lesion likely **began** with a mutation in what genes?
This is asking the progression of the **Ademona--\>Carcinoma sequence** The FIRST mutation is an **APC mutation** (tumor supressor mutation) Incidentally, this mutation is found in all pts with FAP (COcked) AK 53: APC inactivation, COX-2 overexpression, KRAS activation, p53 inactivation
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Pt gets a PE, what happens to the PaCO2, PaO2, pH, HCO3-
The clot that goes to the lung causes a V/Q mismatch. The Q part of this equation decreases causing ischemia to the portion downstream of the clot. This causes a redistribution of blood to poorly ventilated regions. This worsens the V/Q mismatch and causes significant intrapulmonary R --\> L shunting The ensuing HYPOXIA stimulates the respiratory drive, however hyperventilation cannot compensate for the PE fully. This does lead to RESPIRATORY AKLALOSIS. The bicarb will remain the same acutely, but will decrease within 48 hours So pH is INCREASED (7.5), PaCO2 (24) is DECREASED, PaO2 is DECREASED (65) and Bicarb is unchanged (23)
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Pt has cachexia with muscle wasting. What is responsible for the muscle wasting specifically (in the interleukin, interferon cytokine world)?
TNF-alpha is a cytokine that cuases necrosis of some tumors in vitro and produces symptoms of cachexia in experimental animals ## Footnote **\*\*\*TNF-alpha --\> Causes cachexia in malignancy\*\*\***
266
Mucicarmine staining that showed up like this. what is the bug?
**Cryptococcus Neoformans:** a yeast that has a THICK antiphagocytic polysaccharide capsule that is its virulence factor The capsule appears as a clear UNSTAINED zone on India Ink stain and stains RED with mucicarmine. Methanamine silver stain can also be used to identify it
267
When do you see concentric LV hypertrophy vs eccentric LV hypertrophy
**Concentric (**add sarcomeres in parallel)--\> chronic HTN, Aortic stenosis **Eccentric** (sarcomeres added in series) --\> aortic or mitral regurgitation, MI, dilated cardiomyopathy **\*Think eCCentric (the C's are in series)\***
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Drug induced amennorhea?
Drug induced hyperprolactinemia due to **Risperidone** (an antipsychotic drug used in management of schizophrenia) **-Inhibits D1 and D2** -Loss of normal tonic inhibition of prolactin release leading to **hyperprolactinemia** --\> decreased LH/FSH --\> amenorrhea
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Pt has xanthomas with lack of APO-E3 and APO-E4, what is most likely dx, and what is actual mechanism
Familial Dysbetalipoproteinemia is characterized by xanthomas and premature cardiac dz Defect is in APOE3 and APOE4 found on chylomicrons and VLDL that are responsible for binding hepatic apoliporotein receptors Without these two receptors, the liver cannot remove chylomicrons and VLDL from the circulation
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Lipodystrophy is what and what is a likely cause?
Redistribution of fat from extremities to central adiposity is a common adverse effect of HAART therapy (**antiretroviral therapy)** Can be lipoatrophy (loss of subcutaneous fat esp from face) or central adiposity (similar to cushings)
271
Bronze Diabetes seen in what
Hematochromatosis!!! Abnormally high iron absorption that causes iron overload particularily in heart, liver, pancreas See hepatomegaly, abd pain, SKIN PIGMENTATION, DM, impotence, and pigment cirrhosis Usually see \>50% saturation of transferrin and elevated serum ferritin, and elevated serum iron
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Continuous murmur with Inspiratory splitting of S2 is indicative of....?
Patent Ductus Arteriosus Heard best in L infraclavicular region with maximal intensity at S2
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Best way to tell T-cell Acute Lymphoblastic Leukemia from B-Cell ALL?
**B-Cell ALL** is 70-80% of cases **CD10, 19, 20** **T-Cell ALL** is 15-17% of cases. Often presents as a mediastinal mass that presents with SVC symptoms, resp sx, dysphagia **CD2,3,4,5,7,8**
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What is NF-kappa beta? What is relationship with Crohns
proinflammatory transcription factor that increases **cytokine production** In Crohns, there is a NOD2 mutation that DECREASES expression of NF-KB, which impaires the innate immune response in the intestinal mucosa
275
What disease is associated with spontaneous subluxation of a cervical vertebrae causing acute paralysis that gets better with time after the bone stops compressing vertebrae? ESP AFTER INTUBATION
**Long-standing RA** can affect the cervical spine causing vertebral malalignment (subluxation) that can affect the alantoaxial joint This can be worsened by intubation as the pts head is leaned backwards leading to acute compression of spinal cord
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Pt has cardiac defect where they have decreased outward potassium flow and thus prolongation of the AP. What disease and what is likely consequence (ie what will kill him)?
This pt has **congenital long QT syndrome** Prolonged QT predisposes to development of life threatening ventricular arrhythmias (torsades) and can present as recurrent palipitations, syncope, or sudden cardiac death
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**_Bone Metastasis:_** Osteoblastic (3) Mixed (2) Osteolytic (5)
**Osteoblastic:** prostate, small cell lung cancer, hodgkins **Mixed:** GI, Breast **Osteolytic:** Multiple myeloma, non-small cell lung cancer, non-hodgkins, Renal cell carcinoma, Melanoma
278
What is the deal with PAH?
It is **actively filtered** from the blood in the glomerulus and **secreted** by the cells lining the proximal tubule. Thus the calculated clearance can be used to estimate Renal Plasma Flow **\*\*It is filtered and secreted but NOT reabsorbed\*\*** This **filtration process cannot be saturated** (thus neither can excretion) The **secretion** is a **carrier protein mediated process** therefore is able to be saturated. The maximum value of PAH secretion is 80mg/min
279
What is the equation for half life
t=(0.693 x Vd)/CL
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Primary hyperparathyroidism
Caused by parathyroid adenoma in 85% of cases and by parathyroid hyperplasia in the remaining cases Sx: bone pain, renal stones, GI disturbances (bones stones, abdominal groans and psych overtones) The skeletal findings most often involve the corcial (compact) bone in the apendicular skeleton \*\*Subperiosteal resorption with cystic dilation\*\* This shows up like a salt and pepper skull
281
Major side effect of amlodipine and nifedipine
**Peripheral edema**, lightheadedness, flushing, gingival hyperplasia
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Prolonged untreated obstructive sleep apnea can cause what?
**pulmonary hypertension and Right sided HF** Most pts with OSA develop **systemic HTN** due to chronic sympathetic stimulation and elevated plasma norepi levels, also leads to **arrhythmias** (Afib/Aflutter)
283
Branched chain alpha-ketoacid dehydrogenase complex. What is it and what are some cofactors (5)?
This is what is deficienct in MSUD, resulting in accumulation of the branched chain amino acids (Isoleucine, Leucine, Valine), resulting in neurotoxicity that includes seizures, irritability, lethargy and poor feeding Requires 5 cofactors: Thiamine, Lipoate, Coenzyme A, FAD, NAD (Tender Loving Care For Nancy) ...therefore some pts with MSUD improve with high dose thiamine treatment
284
What drug used to treat atonic bladder
Bethanechol (cholinergic agonist)
285
Synthesis of nitric oxide (precursors, location, enzyme)
Happens in the endothelium Arginine + O2 --\> NO + Citruline Nitric Oxide Synthase is the enzyme responsible This then diffuses into the smooth muscle where it activate guanylyl cyclase and increases cGMP
286
Burr Cells and Helmet Cells
seen in microangiopathic anemias and MECHANICAL damage of RBC ## Footnote **Schistocyte example is Helmet Cell**
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Tall and skinny male with small testicles and learning disability. What is it and what are some hormone changes?
This pt has **Kleinfelter Syndrome (47; XXY)** These pts usually have atrophied, hyalinized seminiferous tubules (resulting in low inhibin levels) and damaged Leydig cells ( resulting in low testosterone levels). The lack of feedback causes **excess gonadotrophs (FSH, LH), which also causes increased ESTROGEN** These pts also have increased gynecomastia (and thus increased risk for male breast cancer)
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Gerstmann Syndrome
**_A neurologic disorder characterized by:_** Agraphia Acalculia Finger Agnosia Left/Right Dissociation **\*\*Affects the dominent parietal lobes\*\*\***
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48 year old male presents with epigastric pain after episode of binge drinking. Image shown below. This is a cystic lesion. What is the lining of the cyst?
This is a pancreatic pseudocyst These walls consist of **granulation tissue and NO epithelial lining** Found in **acute pancreatitis** (ususally caused by alcohol and gallstones but can also be from trauma, steroids, mumps, protease inhibitors, NRTIs)
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Zollinger Ellison Syndrome
Caused by Gastrin secreting neuroendocrine tumor (gastrinoma) Commonly located in small intestine or pancreas Presents with multiple peptic ulcers that can be located beyond the duodenal bulb Associated with MEN1
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Peak murmur is seen when in image?
Given the difference in pressure between the aorta and left ventricle, this patient must have Aortic Stenosis. This would be heard loudest during the peak of LV contraction (crescendo-decrescendo), which would correspond to B on the graph
292
What is a Councilman Body
Apoptotic hepatocytes form round acidophilic bodies (pink staining on H&E) that are seen in Hepatitis (most likely C) and Yellow Fever
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2 month brought in because of progressive worsening floppy and poor feeding. Stools every other day and pellet-like. Unable to hold head up on own. Mom immigrated from eastern europe and is 38. What is cause of condition? \*\*BABY was BORN in Europe\*\*
Congenital hypothyroidism is one of the most common causes of preventible intellectual disability. Most cases are due to thyroid dysgenesis or iodine deficiency -Neonates initially have no sx since there is maternal T4, then as this weanes, the sx will get progressively worse T4 is essential for brain development and can result in severe and irreversible intellectual disability Treatment with levothyroxine by age 2 can normalize cognitive and physical development \*The screening of hypothyroidism is performed in the US but NOT all other countries\*\*
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Sickle Cell pt with aplastic crisis following respiratory virus
This is due to a Parvovirus B19 (single stranded DNA virus) infection of erythroid precursors in the bone marrow. This is an aplastic CRISIS just RBC decrease (unlike aplastic ANEMIA that is pancytopenic)
295
What is the end product of the HMP shunt? and what is this product then used to create?
The end product is **NADPH** and this is then used in **Fatty Acid Synthesis** and also the glutathione antioxidant mechanism
296
Where is thiamine a cofactor in the TCA cycle? (2)
The pyruvate dehydrogenase complex and the alpha-ketoglutarate dehydrogenase complex \*If give glucoseto the alcoholic that does not have thiamine, cause rapid depletion of the small amount of thiamine that these pts have and worsen sx. Need to give at SAME TIME\*
297
46 year old went to Latin America to visit and came back with watery diarrhea that turned bloody. Also has erythematous maculopapular rash on chest and abd. What bug caused this?
This pt has Typhoid Fever! This is caused by Salmonella Typhi Salmonella is able to penetrate the gut wall via phagocytosis of M cells and bacteria mediated endocytosis by enterocytes. The bacteria are then able to survive and proliferate within macrophages found in the submucosal region (Peyers Patch) causing severe inflammation and ensuing bacteremia **\*\*See Salmon colored rose spots on abd\*\***
298
pt has hx of renal transplant 8 months ago nd has 3 days progressive retrosternal CP dysphagia and odynophagia. Fever 101. See **linear shallow ulcerations on the lower esophagus.** What is causing this? and what would be seen on tissue bipsy?
This is **CMV esophagitis** which can occur in immunocompromised pts CMV can be transmitted to transplant recepients from the donor CMV esophagitis presents with odynophagia or dysphagia **On tissue bipsy would see enlarged cells with basophilic or eosonophilic intranuclear inclusions**
299
Neck stiffness Tumbling motility (or actin rockets if outside cell) Gram-positive rods What is the bug and what is the source of sickness?
Listeria, usually comes from contaminiated food. Can multiply in cold temperatures (so grows well in refrigerated food) Found in milk products and unwashed raw veggies Tx: ampicillin
300
Class I antiarrhythmics order of Na channel binding strength
1C\>1A\>1B 1A: quinidine, procainamide, dysopyramide 1B: lidocaine, phenytoin, mexilitine 1C: flecainide, propanephone **Think CAB!**
301
Ovarian torsion
Torsion that happens when the **ovary wraps around the infundibulopelvic ligmanet** (suspensory ligament of the ovary). This houses the ovarian blood and nerve supply The main risk factor is having a large ovarian mass (cyst, neoplasm). The weight of the mass causes the ovary to twist and oclude the vessels and nerves Usually present with sudden onset unilateral pain
302
progressive onset of heart failure following a viral illness should raise suspicion for?
Dilated cardiomyopathy caused by viral myocarditis Myocardial inflammation leads to dilation and enlargement of the heart chambers (eccentric hypertrophy) with decreased ventricular contractility (systolic dysfunction)
303
Meneire's Disease
**Meniere's Disease** is a common cause of vertigo due to increased pressure and volume of endolymph. Leads to recurrent vertigo, ear fullness/pain, unilateral hearing loss and tinitis ## Footnote **=endolymphatic hydrops**
304
Cavernous Sinus Thrombosis
Usually caused continuation of spread by infection from the medial third of the face, sinus or teeth by S. Aureus but can also be caused by strep, mucor, rhizopus This results in paralysis of III, IV and VI CN III palsy may also result in ptosis and mydriasis
305
Chlorpheniramine
First gen antihistamine that blocks central and peripheral H1 receptors
306
common tumor markers AFP CA19-9 CA125 Carcinoembryoinc antigen hCG PSA
307
Homeobox (HOX) gene
Highly conserved DNA sequence that is usually 180 BP in length. These genes encode for **transcription factors** that bind to regulatory regions on DNA
308
What part of the brain produces NE and where is it located?
The Locus Ceruleus produces NE It is a pigmented brainstem nucleus located in the posterior rostral pons near the lateral floor of the fourth ventricle It involves mood, arousal (reticular activating system(, sleep-wake cycles, autonomic function (BP control)
309
pt presents with HR 160. He does gentle neck massage below the angle of the right mandible and the HR comes back down. What does he have and why did this help?
He has paroxysmal supraventricular tachycardia (PSVT), which is almost always due to a reentrant impulse traveling between the slowly and rapidly conducting segment of the AV node VAGAL maneuvers such as carotid sinus massage, dumping head in cold ice bucket can be used to actively terminate PSVT This works by increasing afferent firing from the carotid sinus, which increases vagal parasympathetic tone. This SLOWS conduction through AV node and prolongs AV node refractory period
310
What tissues have GLUT that are insulin dependent and what have GLUT that are insulin independent and what type of GLUT are they?
ONLY GLUT 4 is responsive to insulin\*\* this is expressed in skeletal muscle and adipocytes GLUT1 --\> erythrocytes GLUT2 --\> hepatocytes, pancreatic B-cells, basolateral membrane renal tubules GLUT3 --\> placental, neuronal GLUT5 --\> fructose transporter
311
MEN2B 3 things and what associated with?
1. Pheochromocytoma 2. Medullary carcinoma thyroid 3. Mucosal neuromas (oral/intestinal ganglioneuromatosis) **\*\*Marfanoid body habitus** Mutation in RET gene
312
How to test for concentration (and comprehension, short term memory, long term memory, language, visual spacial, executive functioning)
months of the year backwards
313
bat droppings in a cave?
Histoplasmosis oval cells within macrophages
314
High potency vs low potency antipsychotics (first gen)
These are effective for schitzophrenia and psychosis **Low potency --\> (chlorpromazine, thioridazine)** cause non-neurological side effects (histaminergic, cholinergic, noradrenergic) **High potency --\> (haloperidol, flufenazine)**cause neurological side effects (extrapyradimal) due to potent D2 antagonism in nigrostriatal pathway
315
Label the arrow and everything you see
316
**Generalized pruritis** and Vitamin A deficiency (showing up as night blindness) in **44 year old woman** with hyperkeratosis (thickened dry skin) paints picture of what?
**Primary Biliary Cirrhosis** characterized by destruction of small bile ducts in the liver. Pts with PBC develop malabsorption of fat soluble vitamins ADEK This pt also has Vitamin A deficeincy which Can dx PBC with anti-mitochondrial antibody
317
Brown pigment stones in gallbladder
This is caused by E. Coli infection or Helminthic (ascaris lumbricoides, clonorchis (liver fluke)) --\> leads to release of **microbial B-glucuronidase** by injured hepatocytes and bacteria These stones are comprised of calcium salts and unconjugated bilirubin
318
Brief recap of the parasympathetic and sympathetic NT released in regards to preganglionic and postganglionic
319
NE acts where?
**alpha-1--**\> increase IP3 --\> peripheral vasoconstriction **alpha-2** --\> decrease cAMP --\> decrease release NE and insulin **Beta-1 -**-\> increase cAMP --\> increase cardiac contractility **NOT REALLY any action at B2 for NE**
320
pathogenesis of HIV associated dimentia
inflammatory activation of microglial cells Should be expected in AIDS pts with cognitive decline
321
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neurons with shrunken nuclei, no detectable nissl substance, and intence eosinophilic cytoplasm indicate?
**Irreversible cell injury** This becomes visable 12-24 hours post injury and followed by death of the neuron Remants are then phagocytosed by microglia and then see astrocyte proliferation into a glial scar
324
Phyostigmine Pyridostigmine Pralidoxime Atropine
**Phyostigmine** --\> tertiary amine that can cross BBB, used to treat atropine overdose **Pyridostigmine** --\> used for myasthinia gravis **Pralidoxime** --\> used for organophosphate OD (reverses BOTH muscarinic + nicotinic effects but does NOT enter CNS) **Atropine** --\> competitive inhibitor of AChE-I, used in organophosphate OD, enters CNS to reverse muscarinic side effects
325
pt has S3 heart sound, how do you hear this noise the best and where is the best place to hear it?
This is best heard when the pt is in the l**eft lateral decubitus position** at the **end of expiration**. This makes the sound even more audible by decreasing lung volume and bringing the lung closer to the chest wall
326
pt has S4 and bibasilar crackles. Started on Nitro, what happens to: LV End Diastolic Pressure Peripheral venous capacitance Systemic Vascular Resistance
Nitrates are primarily venodilators that increase peripheral venous capacitance, thereby reducing cardiac preload and LV end diastolic volume and pressure. Also have a modest effect on arteriolar dilation LV End Diastolic Pressure --\> DECREASE Peripheral venous capacitance --\> INCREASE Systemic Vascular Resistance --\> DECREASE
327
Regardless of the type of aortic dissection, what is the first step?
Intimal Tear
328
What type of viruses are susceptible to ether and gastric acid (and other organic solvents)?
Enveloped viruses (the envelope is made up of the NUCLEAR membrane, and thus is weak as shit) NON-enveloped viruses are generally resistent to the actions of ether
329
14 year old is pregnant and wants to not tell her parents about prenatal or abortion? which is legal
She can not tell her parents about the prenatal care but NOT if she gets an abortion She could also get STD tx without telling her parents, contraception, drug/alcohol tx programs
330
nocturnal cough in the absence of heartburn
**very indicative of GERD** these are considered extraesophageal symptoms (nocturnal cough and hoarsness) in the ABSENCE of heartburn **=silent GERD**
331
Nevirapine, efavirenz
These are NNRTI (nonnucleoside reverse transcriptase inhibitors) that do NOT require activation via intracellular phosphorylation
332
urge incontinence does what to detrusor
OVERACTIVITY! sudden overwelming need to empty the bladder The detrusor muscle remains relaxed to fill the bladder and then constricts to contract the bladder and urinate
333
side effects of ACE-I CATCHH
Cough Angioedema Teratogen Increased creatinine HYPERkalemia Hypotension
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selective COX-2 inhibitor
Celecoxib
335
What is nursemaids elbow?
Radial head subluxation The annular ligament tears from its periosteal attachment to the radial neck. The ligament slips over the radial head into the joint where it becomes trapped
336
mitral valve prolapse where heard and what make less?
mid systolic click followed by mid-late systolic murmur heard at cardiac apex. Heard less when squatting (increased LV volume pulls valve into more anatomical position) Caused by pathologic **deterioration of the connective tissue** affecting the mitral valve leaflets and cordae tendenae Can also be secondary to Marfans, Elers Danlos, Osteogenesis
337
what tissue CANNOT use ketones as a source of energy
erythrocytes! NO mitochondria
338
Prostacyclin (PGI2) --\> Thromboxane A2 --\>
Prostacyclin --\> INHIBITS platelet aggregation and adhesion to vascular endothelium and vasodilates Thromboxane A2 --\> enhances platelet aggregation
339
Thiazolidinediones
These are the -**glitazone** T2 DM meds they reduce insulin resistance by binding PPAR-Y The main side effect is fluid retention due to increased Na reabsorption in the collecting tubules They also cause adipose wt gain (increased fat storage and increased number of adipocytes)
340
Pt has crohns, likely to also have?
**Dermatitis herpetiformis:** erythematous pururitic papules that often occur on extensor surfaces (they look kinda like herpes simplex breakout) Characterized by microabscesses containing fibrin and neutrophils at the dermal papillae tips Pathogenesis is formation of **IgA antibodies against gliadin in intestine that cross react with epidermal tissue transglutaminase**
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Zinc Fingers
Most commonly identified DNA binding domain in humans (trancription factor motif) Only intracellular receptors located in the nucleus can act directly on transcription factors The molecules that act on these zinc fingers would be ones that act intracellulary **THYROID HORMONE** is an example (also estrogen, aldosterone, cortisol, fat soluble vitamins)
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Congenital hydrocephalus
Hydrocephalus is either due to overproduction or under-excretion Congenital is due to a structural disorder that causes impaired CSF drainage (aqueduct stenosis, Dandy Walker Malformation, Chiari Malformations) Infants with congenital hydrocephalus develop macrocephaly and muscle hypertonicity and hyperreflexia from UPPER motor neuron lesion
343
ADHD drug and mechanism
Methylphenidate and amphetamines and they increase DA and NE and block NE and DA reuptake at synapses in prefrontal cortex
344
Pt has 226 CGG trinucleotide repeats on Chromosome -X What do they have and what is the cause of the condition?
They have **Fragile-X syndrome**, the most common inherited cause of intellectual disability Caused by mutation in long arm of the X-chromosome A "Full Mutation" is characterized by **\>200 CGG** trinucleotide repeats which causes **FMR1 HYPERMETHYLATION**
345
Pupillary light reflex
Shine light into eye and see response in that eye (direct) and in the opposite eye (consensual) The OPTIC nerve is responsible for the AFFERENT loop of this reflex The OCCULOMOTOR nerve is responsible for the EFFERENT loop of this reflex
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CNIII palsy
A somatic component innervates inferior, superior, and middle rectus, inferior oblique and levator palpebrae muscles **Ptosis** occurs due to paralysis of the levator palpebrae muscle **Down and Out** occurs due to the unopposed lacteral rectus (VI) and superior oblique (IV)
347
Demylinating lesion in LEFT optic nerve. What happens when shine light into L eye R eye
**Light into L eye --\> NO change in R pupil, NO change in R pupil** **Light into R eye --\> Constrict L pupil, Constrict R pupil** Remember that the AFFERENT pathway is the optic nerve and the EFFERENT pathway is the occulomotor nerve. In this pt, there is damage to the optic nerve but not anything else **(motor pathways are intact, sensory are not)**
348
what part of the brain atrophies with alzheimers
hippocampus
349
Wolf Parkinson White Triad
**-shortened PR interval** **-Wide QRS** **-delta wave** There is a pre-excitation pathway that excites the ventricles before the AV node, this results in a shortened PR interval with an early upslope at the start of each QRS, thus making each QRS wider than normal
350
67 year old Pt has tibial lumpy protuberance as well as decreased hearing Pathologist finds over 100 nuclei/cell (multinucleated cells) What does this pt have and what makes these cells differentiate
This pt has Pagets Disease of the bone Initially high osteoclast then high osteoblast (common to see hearing issues). See high bone turnover in these pts Osteoclasts in pagets tend to be very large and can have \>100 nuclei The two most important factors for osteoclast differentiation is: 1. macrophage colony stimulating factor (M-CSF) 2. receptor for activated nuclear factor Kappa-B ligand (RANK-L) \*note that osteoprotegrin competatively binds RANK-L and prevents its binding to RANK
351
primary side effect of Statin and what makes this worse (another med given to these type of pts)
**Myopathy** This is made worse with **gemfibrozil** (impair hepatic clearance of statins and lead to excessive blood levels)
352
HbC disease
Glutamic acid --\> Lysine (very basic POLAR) mutation in beta globulin. There is NO hydrophobic interaction (like seen in sickle cell glu --\> val, so NO sickling) Causes extravascular hemolysis See target cells on smear Pts with HbSC have milder disease than HbSS
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Tay-Sachs
cherry red spot NO hepatosplenomegaly accumulation of GM2 ganglioside due to B-hexosaminidase A deficiency progressive neurologic deterioration as these glycolipids accumulate in the brain pts die by age 2-5
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which is more likely to drain to the popliteal lymph nodes and the inguinal lymph nodes? Right great toe Right lateral foot
Right lateral foot
355
skin biopsy of EYELID is below. what is it caused by?
This is an xanthelasma, a cutaneous lesion found on the eyelid that contains lipid-layden macrophages Classically associated with primary or secondary hyperlipidemia Chronic cholestatic processes such as obstructive biliary lesions and primary biliary cirrhosis result in hypercholesterolemia and result in xanthelasma
356
T2DM most likely cause of death
MI
357
chorioretinitis intracranial calcifications hydrocephalus
Toxoplasmosis infection (caused by cat poo) It is a TORCH infection but the fetus is only affected if the mother is infected within the first 6 months of pregnancy
358
what do integrins do?
family of transmembrane protein receptors that interact with the extracellular matrix by binding to specific proteins such as COLLAGEN, FIBRONECTIN, and LAMININ
359
where do anal fissures happen most often?
posterior midline distal to dentate line likely due to poor blood supply anal fissures are due to stretching of the mucosa and are characterized by longitudinal tears in the anal canal distal to the dentate line Most are due to high anal pressures and passage of hard stool, or MSM, or diarrhea
360
what is the effect of verapamil on the cardiac action potential?
block calcium channels in the cardiac slow-response tissues, which SLOWS phase 4 diastolic depolarizaiton and conduction velocity and the SA and AV nodes Verapamil and Diltiazam are used to prevent PSVT
361
At physiologic conditions (resting membrane potential -70), what type of ions would flow OUT of cells K Na Cl Mg Ca
ONLY K would flow out if allowed to and if the resting membrane potential was -70
362
what is seen in this ECG and what does it indicate?
There are delta waves, which indicate **Wolf Parkinson White syndrome** where there is an AV accessory pathway The triad is: **short PR** **wide QRS** **delta wave**
363
inheritance of G6PD
X-linked recessive
364
face grimacing, lip smacking, and twisting movements of fingers?
**Tardive dyskinesia** associated with long term dopamine antagonist medications (antipsychotics)
365
Pt has aortic stenosis and then develops AFIB (10% of pts do), and then develops Pulmonary Edema, why?
They have loss of atrial contraction due to the AFIB, which can lead to decreased PRELOAD in the LV. This can also lead to backwards flow into the lungs leading to acute pulm edema
366
Left temporal hemiretina flame lesion. This travels through to what part of the thalamus
The LEFT lateral geniculate body in the thalamus The left temporal hemiretina receives visual information from the nasal visual field of the LEFT eye
367
Phenelzine, mechanism and when to use it?
This is a MAO-I that are antidepressants that work by blocking the breakdown of NA, DA, 5HT Useful in treatment-resistent depression (atypical subtype of major depression) Atypical depression --\> mood reactivity, leaden paralysis, rejection sensitivity, increased sleep and appetite
368
tingling in face following thyroid surgery?
This is Chovstek sign (hypocalcemia) due to thyroid surgery where they accidently removed the parathyroid glands and thus decreased PTH, thus do not have adequate calcium levels
369
pt had bloody diarrhea a week ago and currently presents with acute renal failure CBC shows anemia and thrombocytopenia and shistocytes on smear. WHY
This pt has hemolytic uremic syndrome caused by shiga-like toxin of e.coli 0157:H7 The smear shows shistocytes which are indicative of **--\> microangiopathic hemolytic anemia** (HUS, TTP, DIC, malignant HTN) \*NOTE that HUS and TTP lie on a spectrum \*\*THIS IS NOT AN AUTOIMMUNE PROCRESS\*\*
370
what effect does esmolol have on the ECG reading
Prolong PR interval (the period from the beginning of atrial depolarization (start P) to the beginning of ventricular depolarization (start QRS) Beta blockers have NO effect on QRS or QT interval duration
371
Uticaria
=hives, caused by transient hypersensitivity characterized by intensely pruritic, raised, erythemartous plaques =wheals Most often caused by IgE mediated degranulation of mast cells The **uticaria itself is due to increased permeability of the microvasculature leading to edema of the superficial dermis** **If the deep dermis and subcutaneous tissue was involved, this would be called ANGIOEDEMA**
372
what DM drug increases insulin sensitivity in peripheral tissue
Glitazones ...also increase fatty acid uptake, increase adiponectin production, increase insulin sensitivity
373
Milrinone
A selective **phosphodiesterase-3 inhibitor** that is occasionally used as an **inotropic agent** in pts with refractory heart failure due to left ventricular systolic dysfunction ALSO has PDE-3 inhibiton in the **vascular smooth muscle** can also cause systemic **arterial and venous dilation**, leading to decreased BP
374
34 year old male low grade fever, anorexia/nausea, dark colored urine, RUQ pain upon returning from trip to Mexico?
Hepatitis HAV is most likely would see hepatocyte necrosis if did liver biopsy. This is characterized by cellular swelling and cytoplasmic emptying (ballooning degeneration) likely caused by ATP depletion This is different from Hepatocyte apoptosis where you see councilmann bodies --\> cellular shrinkage and nuclear fragmentation with intense eosinophilia (likely caused by mitochondrial oxidative damage)
375
Catecholamine synthesis, most specifically, what catalyzes NE --\> E
tyrosine (tyrosine hydroxylase) DOPA (DOPA decarboxylase) DA Dopamine B-hydroxylase) NE (PNMT) EPi
376
CREST syndrome, what does stand for and what happens in vessels specifically
Calcinosis, Raynauds, Esophageal dysmotility, Sclerodactylitis, Telangectasias this can be detected with **anti-centromere antibody** This is due to increased COLLAGEN deposition in the tissue trigggered by increase in monoclonal T-cells that secrete variety of cytokines including TGF-beta **See intimal thickening of pulmonary arterioles**
377
porphyria cutaenous tarda (PCT)
the most common disorder of heme synthesis causes photosensitivity, which is thought to be due to the accumulation of porphyrinogens that react with oxygen on excitation by UV light Due to deficiency in urophorinogen decarboxylase see tea colored urine too along with the blistering cutaneous photosensitivity
378
AAA cause
this is an abdominal aortic aneurysm that occurs BELOW the renal artery Risk factors are age \>60, smoker, male, fx Leads to oxidative stress, vascular smooth muscle apoptosis, and **chronic transmural inflammation** of the aorta
379
pt has stomach ulcers, want to give him a COX inhibitor. What one to give?
COX2, no effect on gastric mucosal protection (COX 1 makes prostaglandins that protect gastric mucosa)
380
Anastrozole (given to breast cancer pt) with ER positive breast cancer
Aromatase inhibitor This slows the progression of ER positive tumors
381
angiomyolipoma
benign tumor comprised of blood vessels, smooth muscle, and fat Associated with **Tuberous Sclerosis** **--\> get brain hammartomas and ash-leaf skin patches**
382
what recognizes stop codons and terminates protein synthesis?
releasing factor. they facilitate the release of the polypeptide chain from the ribosome and dissolution from the ribosome mRNA complex
383
pt has gallstone and does not want surgery, what treatment can be offered?
bile acid supplmementation (ursodeoxycholic acid) This pt has a cholesterol gallstone, the most common type of gallstone Administration of hydrophilic bile acid reduce cholesterol secretion and increase biliary acid bile concentration. This improves cholesterol solubility and increases gallstone disolution
384
Xeroderma pigmentosum
Nucleotide excision repair disorder the thiamine dimers made by UV damage are normally repaired by UV endonucleases (this is deficient in these pts)
385
pt has episodes of syncope while buttoning his tight shirt collar, why?
This patient has carotid sinus hypersensitivity, triggered by pressure on the carotid sinus by the tight neck The carotid body chemoreceptors have a **afferent** limb that travels to the medulla via the Herring nerve, a branch of the **glossopharyngeal** nerve. The **EFFERENT** limb carries its signal by the **vagus** nerve
386
Diptheria vaccination causes what body response?
production of circulating **IgG antibodies** against the exotoxin B subunit, effectively preventing disease ## Footnote **--\> IgG against circulating proteins**
387
Kuppfer Cells
hepatic macrophages that line the walls of the sinusoids and participate in RBC breakdown
388
repaglinide
Part of the meglitglinide class of T2DM drugs. Binds to the K channels on B-cell membrane (at different site than sulfonureas) and cause release of insulin. These can also lead to hypoglycemia along with sulfonureas They bind and close the ATP dependent K channel
389
before starting SSRI for pt with depression, what must you screen them for?
manic episode, if they had this they do NOT get an SSRI. This means they have bipolar disorder ## Footnote **If the SSRI is started, can ppt MANIA**
390
pt is in adrenal crisis because they failed to take their levothyroxine for a few days. What drugs must you give them?
Adrenal crisis is characterized by severe hypotension and refractory shock ## Footnote **In addition to aggressive fluids and glucocorticoids (hydrocortisone or dexamethasone)**
391
12 year old boy with instability and gait ataxia comes in. MRI below, what does he have?
**Pilocytic astrocytoma:** these are low grade tumors that arise from astrocytes. The cerebellum is the most common location, but they can also develop within the cerebral hemispheres. Usually appears on MRI as mass with **both solid and cystic components** This is **NOT a medulloblastoma**, the second most common tumor in a child. Although these are the most common malignant tumor in children. They are located exclusively in the cerebellum. These tumors **ARE ALWAYS SOLID ON IMAGING**
392
Defiency in what vitamin in CF pt causes squamous metaplasia?
Defieincy in Vitamin A may cause squamous metaplasia of the epithelial lining of the pancreatic exocrine duct
393
DDAVP for bleeding
increased endothelial protein release (increased vWF)
394
pituitary adenoma (prolactinoma) with resulting GnRH, LH and Testosterone levels?
DECREASED ALL The increased prolactin decreases GnRH, which decreases all of the rest downstream
395
use of niacin as treatment
**lowers VLDL and raises HDL**
396
HLA-B\*5701 mutation, cannot give what HIV med
Abacavir
397
398
what does insulin do when it binds its receptor
autophosphorylates, activates tyrosine kinase, then can go down two pathways P13K pathway --\> glycogen, lipid,
399
Thrombotic Thyrombocytopenic purpura pathophys
Inhibition or deficiency of ADAMTS13 (vWF metalloprotease) ## Footnote **\*von-Wilebrand cleaving factor\***
400
chronic lithium toxicity can be precipitated by what diuretic?
Thiazide, ACE-I, and NSAIDS
401
Most important mediator of sepsis?
TNF-alpha...also IL-1 and IL-6
402
Wallerian Degeneration
Segment of axon that has lost contact with the cell body First swelling and irregularity are noted in the distal segment of the axon The changes seen on the neuronal body after the axon is severed is called **axonal reaction** and the cell body becomes swollen and round, with nucleus displacement to the periphery. Nissl substance becomes fine, granular and dispursed throughout the cytoplasm
403
Where in the brain is the vomiting center?
The chemoreceptor trigger zone is located on the dorsal surface of the medulla at the **caudal end of the 4th ventricle** in the area known as the **area postrema** This area receives blood from the fenestrations in the BBB, which allows it to sample chemicals circulating in the blood
404
pt has end stage renal disease and presents for hemodyalisis and has uncontrolled bleeding at the site of venous catheter insertion. What are the lab PT, PTT, bleeding time, platelet count?
Excessive bleeding is common in pts with significant renal dysfunction due in part to the accumulation of uremic toxins in the circulation. These toxins impair platelet aggregation and adhesion resulting in a qualitative platelet disorder characterized by **PROLONGED BLEEDING TIME** **PT, PTT, platelet count all NORMAL** **Can be improved with dialysis as it removes the toxins and partially reverses the bleeding abnormality**
405
pt presents with reccurent jaundice, elevated LDH, and indirect hyperbillirubinemia suggests.... Also, when RBC are put into hypotonic solution with glycerol, they release Hb, at risk for what?
The pt has **hereditary spherocytosis** Spherocytes demonstrate increased osmotic fragility These pts would be at **higher risk for pigmented gallstones (a complication of any hemolytic anemia)** Pts with HS also present with aplastic crisis due to Parvovirus B19 infection
406
bipolar 1 vs bipolar 2 vs cyclothymic
Bipolar 1 is manic episode +/- hypomanic and depressive Bipolar 2 has hypomania and depressive episode \*Remember that mood usually returns to baseline between episodes Cyclothymic: milder form of bipolar lasting \> 2 years
407
what is going on in this picture. pt is septic and comes into hospital?
He is just getting IV fluid
408
terazosin doxazosin
alpha 1 antagonists that can cause orthostatic hypotension (used to treat BPH, HTN)
409
When is RAS active?
**When it is bound to GTP** It is inactive when it is bound to GDP Activated RAS then begins a phosphorylation cascade that results in the activation of MAPkinase, which enters the nucleus and causes transcription
410
yeast in Great Lakes region
Blastomyces Blasto Buds Broadly
411
normal glomerulus vs pts glomerulus of 12 year old boy brought in with fatigue. Has small protein in urine, RBC casts (pts above, normal below)
His glom is hypercellular typical of PSGN **hematuria, proteinuria, and urine RBC casts --\> PSGN** This pt would have a LOW C3 in the serum (IgM, IgG, C3)
412
Colchicene side effect
diarrhea
413
livedo reticularis is what and what is it seen in?
this is skin mottling that if seen following an invasive vascular procedure is sugestive of atheroembolic disease If you took a look at this patient's kidneys, would see cholesterol clefts in the arterial lumen
414
Emergency cricothyrotomy, what layers do you cross?
superficial cervical fascia, and cricothyroid membrane \*\*DO NOT CUT CARTILAGE\*\*\*
415
left vertebral artery shows retrograde flow instead of antrograde, what is this called and why?
This is called **subclavian steal** and is caused by severe stenosis or occlusion of the CONTRALATERAL subclavian artery symptoms are arm ischemia and bouts of vertebrobasillar insuffiency leading to occasional bouts of dizziness
416
what is wrong with this diagram? pt is in obvious heart failure but not know which valve is affected?
This has a V-wave that is indicative of **mitral regurgitaiton**. The left atrium pressure is higher than it normally. In that diagram we see a 10 mmhg increase in LA pressure that should not be there normally to to backflow leak
417
methemazole (for thyroid)
blocks thyroid peroxidase which then inhibits coupling of iodotyrosines
418
RA drug that causes liver enzyme elevation
**Methotrexate** side effects: - hepatotoxicity - mucositis (mouth ulcers) - pulm fibrosis - myelosuppression (reversible with Leucovorin)
419
potassium perchlorate
antithyroid used to treat hyperthyroidism competitive inhibitor of Iodine at the sodium-iodine symporter
420
regular what has been associated with lower rates of colonic adenoma and adenocarcinoma
ASA (specifically, COX-2) Remember in the adenoma to carcinoma sequence, increased COX-2 overexpression helps this process progress COCKED AK 53 COX2 --\> APC --\> KRAS --\> p53
421
Pringle Maneuver
clamping the **hepatoduodenal ligament** --\> thus clamping the portal triad (common bile duct, hepatic artery, hepatic portal vein)
422
memory loss of recent events but can remember childhood memories
Alzheimers --\> neuritic plaques (picutre) also have excecutive dysfunction and visuospatial impairment Classic neuritic plaques and neurofibrilary tangles (in the neuronal cytoplasm with aggregates of hyperphosphorlyated tau, which normally mediates microtubule stabilization)
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enlarged RBC with that appear blue on Wright Giemsa Stain in setting of hypochromic microcytic anemia.
The enlarged cells are Reticulocytes, which are enlarged RBC. It lacks a nucleus but retains a reticular (mesh-like) network of residual ribosomal RNA
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Down syndrome is due to ___ that occurs during what phase ___ maternal/paternal
nondisjunction that occurs during Maternal Meiosis 1
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acalculous cholecystitis
cholestitis in the absence of a stone. Happens in critically ill pts and is a very poor prognosis
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pt has a stone in the middle of the right ureter, what is seen in his urine Ca and serum Ca
Calcium stones represent 75-80% of all renal stones hypercalciuria is seen These pts remain normocalcemic due to intact regulation of calcium by Vitamin D and PTH
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newborn screening, want high sensitivity/specificity?
Want a high sensitivity so that you cast a broad net and that a negative test rules out the possibility. Then could do a more specific test Sensitivity, a Negative result rules OUT a dx **(SnNOUT)**
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Presbyopia
starting around age 40-50 individuals stop being able to focus on close up objects This is caused by progressive denaturation of the lens proteins and changes in lens curvature causing the lens to become less elastic and lose its accomodating power
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heart defect in Turner
Bicuspid aortic valve
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pregnant lady has normal bp sitting, standing but then decreases when supine?
This is caused by decrease venous return It is called supine hypotension syndrome Supine --\> compression of IVC --\> reduced venous return --\> reduced preload --\> decreased CO --\> hypotension \*predominently occurs in women \>20 weeks and is due to gravid uterus compressing on the IVC
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Bladder cell carcinoma, what is most important prognostic indicator?
Bladder cell carcinoma arise from transitional epithelial cells lining the bladder Present as painless gros hematuria **\*Tumor stage is the most important factor determining prognosis and depends on degree of invasion into bladder wall\*\***
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diabetic pt collapses, what is best thing to give them (assume you have everything)
Glucagon Can be injected intramuscularly or subcutaneously
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pet puppy had diarrhea 1 week ago and now pt has diarrhea, fever, abd pain? what bug
Campy can be from domestic animals or contaminated foods
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20 pack year smoking hx, heavy ETOH use, what is this picture representing and what disease causes this condition?
This pt has clubbing, and this can be caused by bronchiectasis Clubbing can be seen in: CF, large cell carcinoma, TB, broniectasis, pulmonary hypertension, all the classical congenital heard disorders (esp Tet), bacterial endocarditis, Crohns, UC
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basics of bulimia
binge eat, compensatory behavior (vomit or exercise or stop eating for period of time), excessive worry about body shape and wt, MAINTAINS NORMAL body wt \*IF super skinny, probably anorexia
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DDAVP action outside of central DI
Increases release of **vWF** and **Factor VIII** from endothelial cells Also can be used to treat enuresis (but this is a CNS effect)
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Vitamin E deficiency
Presents very similar to B12 defieincy (but without the megaloblastic anemia) Also presents very similar to Fredreich ataxia (GAA trinucleotide repeat) Often occurs in pts with fat malabsorption or abetalipoproteinemia See hemolysis and neurologic dysfunction (posterior column and spinocerebellar tract demylination)
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germ tubes
Candida when grown at 37 degrees C Note that if an immunocompromised pt has fever, night sweats, wt loss (ie picutre of TB) and performs a sputum stain that shows Candida, this came from the ORAL CAVITY Systemic candida is seen in immunosuppressed pts (neutropenic), unlike esophageal that is seen in T-cell mediated immunosuppressed pts like an HIV pt
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Most of water reabsorbed where in kidney
**PCT** 60% (ADH and aldo and those other things play a role, but the majority of water reabsorption comes from the PCT)
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quick facts about crohns
mouth to anus skip lesions cobblestones fistulas transmural inflammation perianal skin tags
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asthma/copd breath at --\> rate Restrictive lung diseases breath at --\> rate
Asthma/COPD breath at lower rate (slow deep breaths) Restrictive lung dieases as well as ARDS, pulm edema breath at rapid and shallow breaths \*Pts with stiff lungs (restrictive) do best breathing at rapid, shallow breaths to minimize the work of breathing. These pts have incresed elastic resistance **Restrictive --\> higher elastance --\> increase RR**
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pernicious anemia
CD4 cell mediated immune response against parietal cells (autoantibodies against parietal cells also seen) See loss of parietal cell mass due to auto-destruction **Decreased INTRINSIC FACTOR** **IMPAIRED B12 absorption** Decreased acid secretion by these parietal cells causes a feedback on the gastric cells to **increase secretion of gastrin** (which are supposed to increase acid release by parietal cells)
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Identify the levels of the spinal cord by photo
Remember that moving rostrally, the amount of white matter increases and the sections become more ovoid Also remember that a feature unique to thoracic is the presence of lateral horns
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etoposide | (think damsel in distress)
**blocks toposiomerase II** induces transient breaks in BOTH strands of DNA used in testicular cancer and small cell carcinoma of lung **STAY IN S phase or G2 phase**
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Bosentan | (think boss stan)
This drug **competitively antagonizes ENdothelin-1 receptors --\> decreased pulmonary vascular resistance** USED to treat pulmonary hypertension (also can use sildenafil, epoprostenol, iloprost)
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Reid index
This is used in chronic bronchitis to assess the thickness of the mucosal gland layer to the thickness of the wall between epithelium and cartilage The Reid index is normally 0.4 The higher the number, the worse the bronchitis In the image below, the reid index would be B/(A+B+C)
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Chiari 1 vs 2
**Arnold Chiari Type 1** is the most common. It is mostly asymptomatic. It is the downward displacement of cerebellar tonsils thru Foraman magnum. Since this is mild, some people are not aware they have this. **Arnold Chiari Type 2** This is the downward displacement of cerebellar vermis and medulla thru Foraman magnum. Also compression of iv ventricle leading to obstructive hydrocephaly. Frequent assoication with syringomelia. Frequent lumbar mengingomyelocele.
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What are the three types of nevi
1. **junctional** --\> **flat** macules. Limited to dermoepithelial junction. **FLAT** **2. compound** --\> both dermal and epidermal components. **GOES DOWN INTO DERMIS** **3. intradermal --**\> **papular**. lose tyrosinase activity and lose pigment
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Where does the phrenic nerve arise from? and what does irritation of this nerve cause?
C3-C5 Lung cancers can frequently invade the phrenic nerve Irritation of the phrenic nerve causes DYSPNEA, HICCUPS, referred pain to the shoulder
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Haldane effect (hemoglobin)
When new O2 arrives in an RBC, it binds to a heme group, this causes increased CO2 and H+ unloading from the RBC \*\*The actual heme is releasing H+
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psuedogout made up of what kind of stones?
calcium pyrophosphate The knee joint is involved in \>50% of pseudogout cases The crystals are Rhomboid Shaped Calcium Shaped Pyrophosphate
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inability to oxidize very long chain fatty acids (VLCA). This defect is where?
Peroxisomes metabolize very long fatty chain acids by beta-oxidation
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med changes a protein from alpha-helix to beta-pleated sheet. What allows this conformational change?
**Primary:** sequence of AA linked by covalent bonds **Secondary:** Alpha or Beta linked by HYDROGEN BONDS. In alzheimers, lose beta amyloid loses its alpha helical structure and forms beta pleated sheets, which are less soluble and prone to aggregation. Aggregates of beta pleated sheets are the primary component of the extracellular (neuritic) plaques seen in Alzheimer. **Tertiary:** 3D protein made up of many secondary
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Rameltadon
melatonin agonist
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Potency of inhaled anesthetic is determined by?
**Minimum alveolar concentration** is the percentage of anesthetic that renders 50% of patients unresponsive to painful stimuli. Corresponds to ED50 on the dose-effect curve Potency is INVERSELY PROPORTIONAL to MAC The lower the MAC, the more potent the drug
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types of infections seen in pseudamonas pts?
- Otitis EXTERNA - Pneumonia (esp in Cystic Fibrosis pts) - Infections in burn and neutropenic pts - Hot Tub Folliculitis - Ecthyma Gangrenosum **\*Remember, this is a MOBILE, gram - rod that is aerobic, and NON-lactose fermenting, oxidase-positive, that produces pyocyanin pigment and produces endotoxin A that inhibits EF-2**
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If I want to dilate the eye, which alpha receptor should I hit?
**Alpha 1 AGONIST** **Phenelephrine** (used to dilate eyes before eye appts) this stimulates pupillary dilator muscles, resulting in mydriasis
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what cells control iron storage and release in the body?
Hepatic parenchymal cells produce HEPCIDIN, an acute phase reactant that acts as the central regulator of iron hemostasis High iron --\> increase hepcidin Hepcidin works via action at ferroportin either on ENDOTHELIAL CELLS or MACROPHAGES
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Lesion here, what actions will be decreased in this pt?
This is a lesion of the anterior cerebral atery. Each ACA supplies the medial region of the ipsilateral hemisphere Lesions here would affect the contralateral FOOT and LEG
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Where is the AV node on the heart?
the Ineratrial septum near the opening of the coronary sinus
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What is the best indicator of the severity of Mitral Stenosis?
The interval from the A2 to the OPENING SNAP As this interval gets SHORTER, the mitral stenosis is WORSE because the valve is opening more forcefully,
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Before starting metformin, need to check what?
Serum Creatinine Can cause lactic acidosis, need to check serum creatinine before starting
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Consequences of long term topical steroid use?
Atrophy, thinning of the dermis that is associated with loss of collagen, drying, cracking, tightening of the skin, telangectasias, ecchymosis
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Inclusion (I-cell) disease
AR lysosomal storage disorder, occurs due to defects in protein targeting Failure of golgi to phosphorylate mannose residues (decreased mannose-6-phosphate) Proteins are secreted extracellularly instead of secreted to lysosymes Results in Coarse facial features, Corneal Clouding, Restricted joint movement, and high plasma level of lysosomal enzymes
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SLE in the heart SLE in the kidney
In the heart see Libman-Sacks endocarditis that is that is thickened valve leaflets with vegetations on BOTH surfaces In the kidney see diffuse proliferative glomerulonephritis, characterized by proliferative and necrotising lesions with crescent formations. See basemement membrane thickening with "Wire-loop" changes
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When is it common to see Carpal Tunnel Syndrome and is it bilateral?
Pregnancy hypothyroidism DM RA **BILATERAL!**
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what are some defining characteristics of a RIGHT SIDED COLON vs LEFT SIDED masses
**Right sided --\>** They grow as exophytic masses. The ascending colon has a large caliber opening so pts usually do **NOT develop obstruction.** -Usually present with **features of IRON deficiency** anemia due to occult blood loss **Left Sided --\>** tend to infiltrate the intestinal wall and encircle the lumen, causing **constipation** and symptoms of intestinal **obstruction**
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This is the bone marrow biopsy on a 7 year old boy with frequent epistaxis, fatigue, easily bruisability Low Hgb Low platelets Low leukocytes
This patient has **idiopathic aplastic anemia** defined by the pancytopenia with bone marrow hypocellularity. The bone marrow biopsy shows an abundance of fat cells and scattered clusters of normal WBC \*this is different from a crisis from a parvovirus B19, which would cause a red cell aplasia only (not pancytopenia)
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when is an S4 heard on a pressure volume loop
S4 is considered an atrial kick. It is heard in late diastole so would be apparent right before the mitral valve closes
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Two types of Galactose metabolism
**1. Galactokinase deficiency --\> deficiency in galactokinase.** **2. Classic Galactosemia --\> galactose-1-phosphate uridyltransferase.** Symptoms are failure to thrive, jaundice, hepatomegaly, infantile cataracts, intellectual disability. BOTH are AR **Classic is WORSE.** Due to accumulation of Galictatol
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Sporothrix Schenckii
thorn prick. dimorphous fungus spreads along lymphatics commonly seen in gardeners Itraconazole is tx of choice
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Transtentorial (uncal) herniation
occurs when the medial temporal lobe herniates between the crus cerebri and tentorium Often leads to occulomotor lesions (fixed and dilated pupil) with "down and out pupil"
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Sputum contains granule-containing cells and crystalloid mass. What cells are these and what interleukin is activated
These are **Charcott-Leyden crystals** largely in response to **IL-5** released by **TH2 cells**
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What is this photo in a 2 year old boy with abd pain and vomiting?
Intussuseption
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PR3-ANCA
Wegner's Granulomatos (granulomtosis with polyangitis)
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aortic arch derivatives 1 2 3 4 6
1st --\> maxillary artery (max=first) 2nd --\> stapedial artery and hyoid artery (s=second) 3rd --\> Common Carotid artery and prox part of internal carotid (C is 3rd letter) 4th --\> aortic arch, prox part of right subclavian 6th --\> prox part of pulm arteries and ductus arteriosus
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Restless leg syndrome
urge to move the legs treat with DOPAMINE AGONIST (ropinerole, pramipexole --\> non-ergots)
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Kozak sequence
seuqence directly upstream from AUG This sequence helps initiate translation at the methionine start codon (AUG) Beta thalassemia can be caused by globin gene mutaitons in this region
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Giant cell arteritis biopsy
Granulomatous inflammation of the MEDIA and involves medium to smaller branches of the carotid artery, esp the temporal artery
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Cilostazol
phosphodiesterase III inhibitor that **INHIBITS PLATELET AGGREGATION and VASODILATES** \* used in internmittent claudication, coronary vasodilation, prevention of stroke or TIA **\*\*Dipyridamole is another drug in this class\*\***
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pt on high dose STEROIDS, take CBC, what number wil be increased and why?
neutrophils because of demarigination of neutrophils previously attached to vessel wall
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talk about the pain migration during appendecitis
starts as **vague abd pain at umbilicu**s due to **visceral pain that enters at T10** As the appendix becomes more inflammed, it irritates the **parietal peritoneum** and abdominal wall and becomes a more **somatic pain** that shifts to mcburneys point
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most common cause of coronary sinus dilation
right sided heart failure due to PULMONARY HYPERTENSION
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polyarteritis nodosa biopsy
segmental transmural inflammation with fibrinoid necrosis medium sized arteries 30% associated with Hep B Rest are idiopathic
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c-myc
transcription activator