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Flashcards in Step 1 Question Review Deck (604):
1

Chronic lymphedema is a risk factor for the development of _______, appearing as firm, violaceous nodules in the right upper arm

Angiosarcoma

2

administration of Mu opioid analgesics can cause ______, leading to spasm and RUQ pain

Contraction of the SM cells of sphincter of Oddi, increasing common bile duct pressures, which can lead to biliary colic

3

blood flow is directly proportional to the _______ (equation)
____ is inversely proportional to the above variable

Vessel radius raised to the 4th power

Resistance is inversely proportional to radius raised to the 4th power

*SO when flow is reduced by a factor of 16, the radius must be half of the size (1/2)^4

4

Overdosing of nitroprusside can cause confusion and lethargy indicative of ______ toxicity. What are other signs of this toxicity?
Administration of a drug that donates _____ can cure this toxicity

Cyanide toxicity (metabolism of nitroprusside can yelped NO and cyanide ions). Cyanide inhibits ox-phosph by binding Fe3+ in cytC. Causes lactic acidosis and bright red venous blood

Sulfur- an enzyme normally transfers a sulfur to cyanide to form thiocyanate to be excreted. Sodium thiosulfate is administered in conjunction with hydroxocobalamin (binds cyanide ions to be excreted) nd sodium nitrite (promotes met-Hb formation which binds cyanide)

5

Haemophilus ducreyi has _______ borders, ____ forming painful ulcers and _____ lymphadenopathy. Diagnosis?
Herpes has multiple, painful ______ ulcers with an ______ and painful lymphadenopathy. Will usually also have:

Ragged borders, pus forming and suppurative (pus) LA. Gram stain of gram negative rods in ‘school of fish’ pattern

Multiple painful vesicular* ulcers/lesions on an erythematous base with lymphadenopathy. Also have dysuria, and systemic symptoms. Tzanck smear or PCR

6

When a patient comes in seeking opioid anagelsics, the most appropriate step by the physician is to

Identify signs of drug-seeking behaviors or prescription misuse- check past prescription history

7

Syndrome in a young, baseball player with an extra cervical rib that presents with ulnar nerve sensory deficits and tingling, numbness and pain of the shoulder and arm as well is likely from compression by what structures

Scalene muscles

Thoracic outlet syndrome- lower trunk and subclavian vessels likely get compressed between anterior and middle scalene and first rib (scalene triangle)

8

Two main players in cardiac myocyte relaxation for removing calcium intracellularly are:

Na/Ca exchanger pump- large extracellular Na gradient to pump out Calcium

Sarcoplasmic reticulum Ca-ATPase (SERCA) actively pumps calcium into the sarcoplasmic reticulum
*ryanodine receptors are involved in calcium RELEASE from SR not sequestration

9

TNF alpha decreases insulin binding activated GLUT4 receptor translocation via what mechanism: activated _____ that causes ______ of beta chain tyrosine kinases on the insulin receptor

What else induces resistance by the same mechanism?

Upregulated serine kinases- phosphorylate beta subunits of TK and renders them inactive even upon insulin binding, disrupting the IP3 pathway by inhibiting that TK phosphorylation

Catecholamines, glucagon, and glucocorticoids

10

Exercise respiratory/CV physiology:
PFTs- increased RR, and _____
Arterial blood shows
Venous blood
CO causes the V/Q to:

Increased oxygen demand causes increased tidal volume->increased minute ventilation

No changes in paO2 or CO2- increased minute ventilation and gas exchange efficacy ensure that there is neither a decrease in O2 or accumulation of CO2
Venous- increased PvCO2, decreased pH
Increased CO and HR= increased pulmonary blood flow that is more evenly distributed throughout the lungs (V/Q ratio from apex to base is more uniform and so decreased physiologic dead space)

11

Minors can consent to things like _______ but not non-emergency medical treatment like ______

Emancipated minors are

Prenatal care, diagnosis and treatment of an STI, contraception and drug or alcohol rehab or emergency care

Not things like abortion- need parental consent or notification in most states

Homeless, a parent, married, military, financially independent, high school graduate

12

What supplies most of the inferior wall of the heart?
What is it a branch of?

PDA- posterior 1/3 of posterior IV septum and inferior wall of LV

Most people have right dominant circulations- PDA comes off of RCA

The inferior surface of the heart is formed by the 2/3 LV and 1/3 RV

13

Pap smear shows an immature squamous cell with dense, irregularly staining cytoplasm and perinuclear clearing, resulting in a halo and enlarged nucleus where the chromatin has condensed as part of apoptosis process
What causes this type of cell presentation?

Koliocytes*- pyknotic nucleus demonstrating raisin like appearance with halo clearing around nucleus

HPV is a ds DNA, non enveloped virus- causing warts or intraepithelial neoplasia

14

A Meckel diverticulum expressing numerous types of tissue like gastric, pancreatic, or endometrial is a an example of what

Ectopy- term that identifies microscopically and functionally normal cells found in an abnormal location due to embryonic maldevelopment

15

Uterine currettage reveals decidualized endometrium with dilated, coiled endometrial glands and vascularized edematous stroma with no embryonic or trophoblastic tissue

Ectopic pregnancy

Changes seen are from progesterone- usually prepares the endometrium for implantation

16

NK cells express _____ and ____ surface receptors
Activated by ____ and _____
Recognize _____

Special characteristics about maturation and antigen specificity

CD16 and 56
Activated by IL-12 and IFN-gamma

Recognize cells with absent MHC I- like virus infected cells or tumor cells

Do not require a thymus so are present in athymic patients
Have no antigen specific activities or memory ability or antigen activation systems

17

Z scores for 95% and 99% confidence intervals, resp is +/- ___ and ____ standard deviation

Standard intervals of standard deviation on normal bell curve=

confidence intervals of the mean can be calculated by:
Standard error=

95% of observations lie within 1.96SDs
99% of observations lie within 2.58 SDs

1 SD= 68%
2 SD= 95%
3 SD= 99%

Mean +/- 1.96x(SD/square root of n) for a 95% CI
SD/square root of n- estimates how far the sample mean is from population mean or variance of different sample means. CI better accounts for the variability due to sampling by including the SE in its calculation

18

Ionizing radiation (gamma and X-rays) induces damage to DNA via _____

Ds DNA breaks and formation of oxygen radicals

19

Opioids are pentapeptides that are derived from ______; similar to _____ and ______

POMC

Like ACTH and MSH

*why there is a close association between the stress HPA axis and opioid system

20

olanzapine is a _______
AE? What do you want to check in a patient

Which drug of this class is associated with QT prolongation? Prolactin elevation?

Second generation anti-psychotic (less extrapyramidal effects)

Metabolic AE- weight gain, dyslipidemia, hypergylcemia, increased risk of DM. Check fasting glucose, lipid profiles, etc.

Ziprasidone; risperidone, resp.

21

Ureteric bud gives rise to the ____
Metnephric mesoderm/blastema gives rise to_____

Collecting system of the kidney- collecting tubules and ducts, major and minor calyces, renal pelvis and ureters

Glomeruli, Bowman’s space, proximal tubules, loop of Henley, DCT

22

8 year old boy with sever dyspnea, tachypnea and inspiratory stridor, dysphagia with solid foods and shows blast cells in peripheral blood smear most likely has ___
What marker would be present

T-ALL- CD 2, 3, 4, 5, 7 or 8+

T cell ALL most commonly presents with mediastinal masses over B-cell ALL (CD10+, 19, 20)

23

Acute pancreatitis related to alcohol abuse as shown by the AST:ALT ratio of >2 and absence of gallstones can be confirmed as alcohol abuse with what lab test?

MCV >100 in the absence of anemia- indicating poor nutrition and folate deficiency, liver disease by direct alcohol toxicity


Hypocalcemia can occur in all types of pancreatitis*

24

State of chronic myocardial ischemia in which both myocardial metabolism and function are reduced to match the coronary blood flow to prevent necrosis. Can show decreased contractility and systolic dysfunction on echo/EF

Brief repetitive episode of myocardial ischemia, followed by reperfusion, protect myocardium from prolonged episodes of subsequent ischemia (multiple episodes of angina before an MI can delay cell death after complete artery occulusion)

Myocardial hibernation (decreased expression and disorganization of contractile and cytoskeletal proteins, altered adrenergic control, and reduced calcium response)- coronary revascularization and restoration of blood flow improved contractility and LV function

Ischemic preconditioning

25

Nitrate free intervals must be provided everyday in order to avoid

Tolerance

Usually patients skip dosing at nighttime- cardiac work is at its least during sleep

26

PAH from proliferative vascularity of the smooth muscle may occur as a hereditary mutation of _____
Treatment?

BMPR2 inactivating mutation (normally inhibits SM proliferation)

Endothelin is a potent vasoconstrictor and promotes SM proliferation and is found in high concentrations in patients with PAH
Endothelin receptor antagonists (bosentan and ambrisentan) alleviate vasoconstriction to lower PAH and improve dyspnea

Can also use prostacyclin analogs or PDE-5 inhibitors

27

The anterior compartment of the leg includes:
(Most common site for compartment syndrome)

Foot extensor muscles, anterior tibial artery and veins, deep peroneal nerve

Damage to this nerve causes decreased sensation between the first and second toes, decreased dorsiflexion of foot, foot drop and claw foot

28

Medicare can cover younger people (<65) who have _____

Medicaid covers

Disabilities, end stage renal disease, or ALS

Homeless, undocumented immigrants, pregnant women and low income families

29

Increased levels of ____ can cause nephrogenic DI (polyuria,polydipsia), weakness, constipation and confusion

Calcium

30

Patient with signs of lung cancer + right sided face and arm swelling, engorgement of subcutaneous veins on the same side of the neck most likely has:

Right sided brachiocephalic vein obstruction

Similar to SVC syndrome but only on ONE side!!!

External jugular drains into subclavian, subclavian joins IJV to form BCV
Right lymphatic duct drains into the BCV

31

RAS proteins exist in two different states
What is bound to them to differentiate these two states and what is frequently mutated in the setting of cancer

What signaling pathway activates RAS

Activated RAS does what

Inactive GDP bound state
Active GTP bound state (has intrinsic GTPase activity that allows hydrolysis of GTP to prevent accumuation- mutations can lead to decreased GTPase)

Growth factor binds TK, causing autophosphorylation

Activated RAS then results in the activation of mito gen-activated protein kinase (MAPK), which enters the nucleus and influences gene transcription

32

A patient with wide, fixed splitting of the S2 has _____
This defect may require repair to prevent irreversible changes in the ________

ASD

Increased blood flow through the pulmonary artery may cause laminated medial hypertrophy that can become so severe it leads to an increased pulmonary vascular resistance higher than systemic. This would then cause a R—>L shunt through the ASD and late onset cyanosis, clubbing and polycythemia

*if pulmonary HTN were corrected, right atrial and ventricular changes could be corrected

33

When data is misclassified due to preconceived expectations or prior knowledge concerning the study. Subjective outcomes*

Non random, inappropriate methods that lead to different attrition rates between groups that reduces the generalizability of the study- like many patients dropping from a trial due to severity of their side effects

Observer bias

Selection bias

34

drugs that high a high ______ and ______ are more likely to be metabolized by the liver, instead of the kidney

High lipophilicity and, hence a higher volume of distribution
Processed to more hydrophilic parts and excreted in bile

*low Vd- confined to the bloodstream and cannot diffuse through cell membrane. Highly protein bound and hydrophilic- urine excretion
Drug that is not well absorbed orally will exhibit first pass metabolism, but won’t be available for hepatic clearance because of low lipophilicity
Low CNS penetration and low rate of resdistribution imply low lipophilicity

35

Elderly female patient presents with a firm, palpable gallbladder showing extensive calcification on CT throughout the gall bladder wall is likely from ______
High risk for_____

Porcelain GB is likely from chronic cholecystitis and found with multiple gallstones

Adenocarcinoma of the GB***

36

Patient with ESRD due to type I DM has excessive bleeding around the IV site when a catheter is inserted into the right IJV before his dialysis treatment
Labs- prolonged bleeding time, normal platelets, normal PT/aPTT
What is the diagnosis?

What other syndrome can have this appearance on LABs?

Uremic platelet dysfunction- accumulation of uremic toxins impair platelet aggregation and adhesion, resulting in a qualitative platelet disorder, characterized by isolated prolonged bleeding time.

Improved with dialysis

vWF deficiency (aPTT could be elevated or normal)

37

Pathophysiology of vitiligo is:

Partial or complete loss of epidermal melanocytes by autoimmune destruction in an area of skin- and therefore a complete absence of melanin

38

What types of adrenergic receptors are on the pancreatic beta cell
What is the predominance and balance/responsiveness of each?

Alpha 2 and Beta 2

Alpha 2- inhibits insulin secretion. This inhibitory response predominates
Beta 2- promote insulin secretion

Epi would activate both receptors, but alpha 2 response is stronger resulting in decreased insulin secretion*

39

_____ and _____ stimulate the RLS step of cholesterol synthesis (HMG COA reductase)

Insulin and thyroxine

40

Most important complication during the recovery phase of kidney tubule damage is ________

Hypokalemia- increased permeability of the epithelium results in hypokalemia primarily which can manifest as muscle weakness and arrhythmias (flat t wave, u wave, st depression and premature contractions)

41

Payor pays a fixed predetermined fee to cover all medical services required by a patient is termed

Insurer pays a provider a single payment to cover all expenses associated with an incident of care

Capitation- like in HMO (vs point of service plans are different in that they allow patients to see providers outside of their network with co-pays and deductibles)

Global payment- for elective surgeries, in which the global payment covers the surgery and any pre-post operation visits

42

What in the primary inhibitor of beta-ox of FA? At what step?

Malonyl-CoA inhibits the carnitine acyltransferase, preventing the transfer of acyl groups into the mitochondria for FA breakdown
Ensures that FA synthesis and oxidation are not occurring at the same time*

Citrate gets shunted from the mitochondrial into the cytosine, broken down into acetyl coa which is then converted to Malonyl coa for FA synthesis

43

Anti-RH(D) antibodies are what isotype of Ig?

IgG, cross placenta and cause lysis of fetal RBCs- IgG form from a prior birth with a blood type of RH+ that crosses into the maternal circulation, causing her to form antibodies that are capable of crossing the placenta and attacking her next fetus

Prevent this by giving a primigravid mother anti-IgG against RhD at the 28th week and during postpartum period to prevent maternal Rh alloimmunization when fetal blood mixes with maternal

44

A complete mole will typically have what genotype?

Paternal 46,XX- duplication of a haploid 23,X sperm

46,XY moles can occur when two different sperm fertilize an empty ovum which is uncommon

45

When an initial embryonic disturbance leads to multiple malformations by disrupting the development of adjacent tissues and structures within that region is called ______
Example: spectrum of structural abnormalities of the brain and face occur due to the incomplete division of the forebrain

Developmental field defect

Holoprosencephaly: this cleavage is supposed to occur at 5 weeks gestation. Failure can result in an array of midline defects* from mild (closely set eyes, cleft lip) to severe (Cyclopes, primitive nasal structure proboscis, midfacial clefts)
This defect is typical of Trisomy 13, sonic hedgehog mutations and maternal alcohol consumption

46

A young, previously healthy patient dies suddenly from a spontaneous intracranial hemorrhage from a cerebral aneurysm rupture. What cardiac defect does he most likely have?

Coarctation of the aorta- usually assoc with berry aneurysms

47

VSZ infection that does not have viral kinases should be treated with what

Cidofovir* a nucleotide or foscarnet a pyrophosphate analog DNA pol inhibitor

Acyclovir, valacyclovir, famcyclovir and gancyclovir all require viral kinase activation to go from a nucleoside to a nucleotide

48

Amphotericin B causes renal toxicity by two mechanisms:
How can renal toxicity cause heart issues/what would you see on ECG

Decreased GFR and direct tubule epithelium toxicity

Nephrotoxicity can lead to anemia and electrolyte abnormalities. Most importantly is hypokalemia, due to increase in membrane permeability of the distal tubule. Hypokalemia can cause weakness and arrhythmias.
ECG of hypokalemia would include flat T wave, ST depression and U waves and premature contractions

49

What affect does estrogen have on thyroid hormone functioning?

Increases TBG which increases TOTAL T4/T3

Increase bound T4 and decreases free T4, which stimulates TSH which then increases T4 available for binding and restores free T4 levels (euthyroid) **why you want to check free T4 when suspecting hyperthyroidism

50

Deletion or addition of a number of bases not divisible by 3 would cause ________; affecting the length of the mRNA transcript

Nonsense mutations occur in exons and affect _______

Frameshift deletions

Affect protein translation* point mutations are unlikely to affect the total mRNA size* (and hence the cDNA length)

51

What two exceptions drain into the deep inguinal lymph nodes instead of superficial?

Glans of penis and posterior calf

52

_____ increases GLUT4 expression on muscle which can cause hypoglycemia in patients with diabetes due to the lack of a _______

Exercise

Counter-regulatory response: no drop in insulin (due to insulin shots) and turning on of glucagon to increase blood glucose

53

An unexpected occurrence involving death or serious injury that requires immediate investigation

Malpractice is _____

Sentinel event- inpatient suicide, death of full term infant, retained object after surgery

Not an actual category of medical errors (near miss, preventable AE, non preventable AE) but a legal term that is a consequence to any type of error that result in harm

54

Angiomatous lesion involving cerebellum or retina, cystic kidney masses indicates what disease? What other findings are associated?

VHL disease- presence of capillary hemangioblastomas (high vascularity with hyperchromatic nuceli) in brainstem, cerebellum, retina or spinal cord. Angiomatosis (cavernous hemangiomas in skin, mucosa and organs, bilaterally RCC, pheochromocytoma

*different from TS: harmatomas of CNS and angiomyoLIPOMAs of kidney. Not lesions that are characterized by high vascularity

*think hypoxia inducible factor defective so increase angiogenesis for my blood/oxygen tumors

55

Probability of independent events being the same=
The probability of at least 1 event turning out differently is given by=

Product of separate probabilities : (95% NPV) chances of 8 people who don;t have the disease as having a negative result is .95^8

Of 1 turning out differently is 1-P(all events being the same)

56

Burn patient is what type of shock?
Skin would appear-
PCWP-
CO-
SVR-
Treat-

Hypotensive shock

Cold,clammy
Decreased PCWP
Decreased CO
Increased afterload
IV fluids- ringer’s lactate (sodium lactate)

57

Shock symptoms with no fever, no response to saline, and high CO, low PWCP, and low SVR is indicative of _________

What is the treatment usually

Early septic shock- **decreased SVR

Isotonic saline- 9%

58

Vaginal bleeding, very elevate hCG and the cervical os is closed with ultrasound showing an empty uterus

Ectopic pregnancy

59

Why does mycobacterium leprae have a predilection for the extremities?

Thrives in cooler temperatures

60

Someone who has a massive PE from DVT is given a drug and a week later is found to have a platelet count of 55,000. This thrombocytopenia was most likely caused by _______

Heparin induced thrombocytopenia- development of IgG ABs against heparin bound platelet factor-4. These complexes activate platelets—> thrombosis and thrombocytopenia


*aspirin doesn’t destroy platelets

61

Teenager with daily headaches, bilateral aching temples, more confused, clumsy with frequent falls and has a broad-based ataxia gait and is slow to answer questions
Toxicity?

Inhaled glue

62

What factor inside muscle cells gets released upon muscle damage and damages kidneys?

Myoglobin break down products damage the kidney cells

63

Epimere forms
Hypomere forms

The dorsal back muscles
The ventral thoracic and abdominal muscles

64

Which area should be avoided to leave the SA node intact?

The junction of the SVC and the right atrium

65

What supplies dentition to the lower teeth?
Branch of what nerve?

Inferior alveolar nerve (dental)- innervate the lower teeth
Branch of the mandibular nerve

66

Tactile fremitus is inc/dec in pneumonia?

In pleural effusion?

INCREASED!

Decreased

67

Child comes in with round shiny bumps in areas where she has eczema, her friend had similar bumps on their play date last week. PE shows firm, smooth, umbilicated papules in clusters. What viral group is this?
What histology is typically seen

Poxvirus- molluscum contagiosum
Guarneri bodies inclusions- sites of cytoplasm replication of viruses
Dumbbell shaped core


*different from varicella- skin lesions all develop at the same rate unlike chicken pox

68

Patient with right cerebral cortex wound from an accident has left lower extremity paralysis- what would the reflex response be?

Hyperreflexia- UMN damage even if paralysis of these muscles

69

Patient has extreme sensitivity to light and exposure to sun causes the formation of vesciles and blisters on the skin. There is an increased synthesis of compounds in the skin that are subject to excitation by visible light. What pathway is most likely deficient?

Heme synthesis- porphyria. Accumulated uroporphyrinogen III

70

Patient with sudden onset of uncontrollable irregular movements of the left side of the body, irregular flailing movements of the proximal appendicular muscles on the left is due to a lesion in ______

Right subthalamic nucleus (contralateral hemiballism)

71

Which test is best to confirm hyperthyroidism in someone who has an elevated serum total thyroxine (T4)

Measure the free T4

T3 is more actively regulated, so small increases or decreases are managed in states of hyper or hypothyroidism

72

What percentage of people fall within the +/- 1 standard deviation

68%
95%- 2 standard deviations
99.7%- 3 standard deviations from the mean

73

Splice sites usually consist of what nucleotide pairs?

5’ GT (u) and AG 3’

74

Loratadine, fexofenadine, desloratadine, cetirizine are all
______ used for ______

Second generation anti-histamines
Used for allergies over first generation- less sedating because of decreased entry into CNS

75

Next best step in management of third degree heart block with a pulse of 40 and BP 92/56 is ______ and administration of ______

Insertion of a venous pacemaker (until a permanent one can be placed) and administration of atropine to increase HR by decreasing vagal activity

76

Adipose brown fat does what to the proton gradient of the ox phosph chain
This does what to the ATP:O2 ratio

Uncoupling agent that increases the permeability of the membrane causing a decreased proton gradient to generate heat
Increased oxygen consumption and ATP synthesis stops

Also aspirin and 2,4 dinitrophenol

77

What things does vitamin B6 help synthesize? (6 things/groups)

ALT/AST
Glycogen phosphorylase reaction
Cystathione/cystine
Heme
Niacin
Histamine and NTS

78

Adipose brown fat does what to the proton gradient of the ox phosph chain
This does what to the ATP:O2 ratio

Uncoupling agent that increases the permeability of the membrane causing a decreased proton gradient to generate heat
Increased oxygen consumption and ATP synthesis stops

Also aspirin and 2,4 dinitrophenol

79

What things does vitamin B6 help synthesize? (6 things/groups)

ALT/AST
Glycogen phosphorylase reaction
Cystathione/cystine
Heme
Niacin
Histamine and NTS

80

How does anion gap acidosis from increased lactic acid occur in sepsis?

End organ hypoperfusion in septic shock impairs tissue oxygenation and decreases oxidative phosphorylation, leading to buildup of NADH and shunting to lactate production
Hepatic hypoperfusion during sepsis also contributes to lactic acid accumulation as the liver is the primary site of lactate clearance/conversion back to glucose

81

The most common cause of congenital bilateral absence of the vas deferens is _____

Cystic fibrosis

82

In the case of no legal documentation or health care proxy, medical decisions fall to _____

Next of kin- spouse

Contact ethics committee if family members cannot agree

83

Gram positive, motile rod that produces a very narrow zone of beta hemolysis that can multiply at refrigerated temperatures is likely eliminated from the body by what immune response?

Cell-mediated immunity (cytotoxic T cells and activation of macrophages)- Listeria monocytogenes is a facultative intracellular pathogen

*avoids antibodies by actin polymerization, forming ‘rocket tails’ between cells

84

Disease characterized by anti-mitochondrial antibodies is characterized by ____ and is biopsy findings will appear most similar to ______

PBC- destruction of small and mid-sized intrahepatic ducts with resulting cholestasis

Shows patchy lymphocytic inflammation with destruction in intrahepatic bile ducts with granulomas and bile staining of hepatocytes

GVHD will most typically affect the liver via lymphocyte infiltration and destruction of small intrahepatic bile ducts

85

Niacin dependent enzymes of the TCA cycle include:

Pyruvate dehydrogenase—> Acetyl CoA
Isocitrate dehydrogenase—>alpha-KG
Alpha-ketoglutarate DH complex —>succinyl-CoA
Malate DH—> oxaloacetate

86

What is the anatomical landmark to help locate the optimal site for lumbar puncture?

Iliac crest
Optimal location of needle is L3-L4 space or L4-L5 space. The L4 vertebral body lies on a line drawn between the highest points of the iliac crests

87

The GF binding and tyrosine kinase autophosphorylation—>PI3K activation—>Akt (Protein kinase B) activation, which will lead to _______ activation and what result?

How is this last step inhibited?

mTOR translocation to the nucleus and gene transcription which induces cell growth and proliferation

PTEN inhibits mTOR by removing a phosphate from PIP3

88

EBV preferentially infects ____ by binding the ____ cell surface receptor
_____ clonally expand in response to this infection, showing atypical cells on blood smear

B lymphocytes by binding the CD21 cell surface receptor

Cytotoxic CD8+ T cells clonally expand to destroy the virus

89

A patient with LVHCM from mutated ____ likely has LV outflow obstruction created by ______ and the IV septum

Sarcomere proteins- protein C and beta-myosin heavy chain

Anterior mitral leaflets- shift toward aortic valve during systole causing eccentric mitral regurg and LVOT

90

Important genes upregulated by thiazolidinediones include ____ and _____
These genes are upregulated by binding of ____

GLUT4 on adipocytes and skeletal muscle to increase uptake
Adiponectin, a cytokine secreted by fat tissue that increases the number of insulin-responsive adipocytes and stimulates FA oxidation

Binding of PPAR-gamma: nuclear receptor that alters the transcription of these genes

91

Recently diagnosed with nephrotic syndrome and then sudden development of abdominal flank pain, gross hematuria, and a left-sided varicocele suggests _______ due to loss of _____ from the prior nephrotic syndrome

Renal vein thrombosis- due to loss of antithrombin III in nephrotic syndrome, resulting in a hypercoaguable state

92

Primary route of copper elimination is via

Secretion into bile by liver and excretion in stool

Liver also regulates copper incorporation into an alpha-2 globulin to form ceruloplasmin and released into circulation in this form

93

Fracture of the _____ bone, region where the frontal, parietal, temporal, and sphenoid bones meet in the skull
What artery is affected? Branch of ?
What can this artery damage progress to?

Pterion

Middle meningeal artery lacerations- epidural hematoma—>uncial herniation with oculomotor nerve palsy

Branch of the maxillary artery, which enters the skull at the foramen spinosum

94

Left cerebellar hemisphere lesion would result in _____ sided deficits in _____, ______, and ______

LEFT- ipsilateral. Connect with lateral descending motor systems

Disdiadochokinesia, limb dysmetria (overshoot/undershoot during targeted movement), and intention tremor

95

Severe chest pain not relieved by rest or nitroglycerin and ECG showing peaked T waves, ST elevation and eventual Q waves in I, aVL and V1-V3 is from
Most commonly caused by

A trans mural MI in the anterolateral LV (prinzmetal’s angina can present like this but would respond to nitroglycerin)

An acute plaque change- rupture which produces a superimposed thrombus that completely occluded the involved coronary artery

*A lesser degree of occlusion superimposed on an acute plaque change would more likely cause unstable angina, subendocardial MI, SCD.
A stable plaque without an overlying thrombus but obstructing over 75% of the artery lumen would likely cause stable angina

96

Risk of wrong site surgery can be reduced by

Dual identifiers (usually a nurse and physician) independently

97

An elevated HbA2 percentage is suggestive of ______

Beta thalassemia minor- becomes elevated in these patients to compensate for beta globin chain underproduction, but the resulting microcytic cells are prone to hemolysis, which can cause a falsely low HbA1C levels

98

vitamin B12 deficiency is likely in which of the following patients?
-patient who underwent a total gastrectomy 8 months ago
-patient who became a vegan 6 years ago

Vegan- it takes 4-5 years to develop B12 deficiency
The total gastrectomy patient would likely eventually develop deficiency, but not after 8 months.

99

during the wound healing process, fibroblast migration and proliferation are controlled by _____ and _____

PDEF and TGF-beta

100

Hospice care requires a survival prognosis of

Less than 6 months

101

Mycoplasma that appear as long parallel chains of mycolic acids represent the presence of ______, which has what activity?

Cord factor- virulence

Surround the organism forming a Micelle and prevents macrophage-mediation destruction within the phagolysosome and helps drive caseating granuloma formation

102

Histological analysis of a _______ in the heart shows scattered cells within a mucopolysaccharide stroma typically with a mid-diastolic rumble heard best at the apex
These produce large amounts of:

Atrial myxoma

IL-6- weight loss, fever
VEGF- angiogenesis, hemorrhaging of the tumor

103

Most anal fissures/tears occur that the ______ likely due to decreased blood flow in this area

Posterior midline- due to passage of hard stool in patients with chronic constipation

*fissures not on the midline are more likely due to unusual causes and warrant further investigation (IBD, malignancy, infection)

104

A severe side effect to aggressive osmotic diuresis, mannitol, in those with increased ICP trauma patients is ______

Pulmonary edema- caused by the rapid rise in volume that increases overall hydrostatic pressure in vasculature. Continued rise in plasma osmolality causes more water and potassium to move out of cells and brain into vasculature, worsening edema.

105

Bugs that cause necrotizing fasciitis- 3

Which one is pyrrolidonyl arylamidase (PYR+) positive

Strep pyogenes- PYR + *replacing the bacitracin test
Staph aureus
Clostridium perfringens

106

Esophageal manometric study of a patient having difficulty swallowing solids and liquids shows periodic, simultaneous and non-peristaltic contractions of large amp/duration

What would barium swallow look like?

Diffuse esophageal spasm from impaired inhibitory neurotransmission- several segments contract inappropriately at the same time, which gives a disorganized non-peristaltic appearance

Barium swallow would show corkscrew esophagus

107

MOA of TCA that causes death induced arrhythmias and anti-cholinergic toxidrome presentation

Inhibition of fast sodium channels, slowing down the myocardial depolarization and leads to arrhythmias- most common cause of death in overdose

108

Reverse transcription PCR is used to detect and quantify levels of what?

mRNA

The DNA template here is generated by the reverse transcriptase on the mRNA sample, producing a cDNA to the mRNA (contains the Exons of a gene and the untranslated regions) that is then amplified by PCR

109

_____ can cause irregular uterine enlargement, which can cause heavy bleeding, reproductive difficulties or put pressure on adjacent organs; like the colon causing constipation
Patients will commonly say they relieve the pressure by ‘splinting’ to defecate, which involve manual deflection of the obstruction

Fibroids- a posterior subserosal uterine leiomyoma in this case

This presentation is similar to a prolapsed posterior vaginal wall but without uterine enlargement and with the feeling that something is “falling out of the vagina”

110

____ and _____ are nuclear transcription factors that directly bind DNA via a leucine zipper motif

______ tests are used to ID and isolated proteins that bind DNA (TFs, nucleases and histones)

C-Jun and C-Fos

Southwestern blotting- proteins separated by gel electrophoresis and transferred to a filter membrane, labeled ds DNA probes are used to detect a specific protein of interest

111

If there is suspicion of abuse, _____ is the next step

Ask permission to interview the child alone
Parental refusal to be interviewed alone is considered concerning for abuse

Then call CPS

112

_____ is an inducible enzyme unregulated during inflammatory reaction by IL-1 and TNF-alpha but is undetectable in normal tissues under normal conditions.
What drug can directly inhibit this enzyme?

COX-2- produces pro-inflammatory AA metabolites (PGD2 and E2: vascular permeability and fever)

Celecoxib

(Glucocorticoids decrease the transcription of COX-2)

113

Dementia therapies target enhanced _____ transmission, neuroprotection via _____ or ______ receptor antagonism

Cholinergic transmission (donepezil, a cholinesterase inhibitor)

Antioxidants (Vit E: alpha tocopherol)

Antagonize NMDA receptors (memantine)

114

_____ is a major pathogen in burn patients

Pseudomonas- wound infections: shows erythema, induration, and gram negative, oxidase positive organisms that do not ferment lactose

115

The imbalance of estrogen and progesterone places patients with PCOS at increased risk for _____ later in life

Endometrial hyperplasia and adenocarcinoma

116

narcolepsy will typically have a decreased level of _____ in the CSF

Hypocriten-1 (orexin-A)- produced by neurons in the lateral hypothalamus

117

Renal scarring/cortical atrophy in the upper and lower poles of the kidney is distinctive of:
What pathology usually underlies the above
If uncorrected, this can lead to:

Recurrent pylonephritis- typically from retrograde flow of infected urine especially in vesicoureteral reflux (ureter enters bladder wall at a perpendicular angle)

Ongoing injury leads to renal scarring, most commonly at the upper and lower poles. Can eventually lead to secondary HTN

118

4 year old boy showing precocious puberty signs and labs show a high serum 17-hydroxyprogesterone and testosterone level likely has:
Treatment

CAH due to 21 hydroxylase deficiency from autosomal recessive defect in the gene

Treatment is to suppress ACTH- since there is lack of cortisol production, ACTH is secreted in high amounts, and this just stimulated more androgen production from adrenals. Give small doses of exogenous corticosteroids to suppress ACTH

119

Patient is prescribed ACE-i, ARBS or diuretics and BP stays elevated and plasma renin is within normal range values indicates

Medication noncompliance

Induction of meds would block RAAS and/or decrease BP, causing increased feedback to increase renin

120

______ is present when the effect of the main exposure on the outcome is modified by the presence of another variable
How can this be differentiated from confounding variables?

Effect modification

Stratified analysis shows the strata to have different measurements of association in effect modification. If confounding variables are present, stratified analysis will show no significant difference between the strata

121

What affect do beta-blockers have on hyperthyroid treatment?

Blunt adrenergic manifestation of hyperthyroidism, but also reduce the conversion of T4–>T3 by binding 5’monodeiodinase in peripheral tissues.
This is also decreased by glucocorticoids and propylthiouracil

122

What congenital heart disease causes cyanosis at birth that improves with squatting, prominent RV impulse and a systolic murmur?
What causes this malformation?

Tetralogy of Fallot- abnormal neural crest cell migration leads to anterior and cephalic deviation of the infundibular septum, resulting in a malaligned VSD and an overriding aorta. Typically causes RV outflow tract obstruction and then RV hypertrophy

*a VSD alone would cause a left to right shunt, which does not present with cyanosis at birth

123

Leuprolide is a ______
Can cause transient rise in ___ and ____ on initial administration

GnRH analog

Transient rises in LH and testosterone production on initial administration

124

What type of breathing pattern is typically seen in patients with
CHF?

Cheyne-stokes breathing- apnea is followed by gradually increasing then decreasing tidal volumes

chronic hyperventilation with hypocapnia induces apnea during sleep when the partial pressure ofCO2 falls below a certain threshold. Apnea will cause excessive buildup of CO2 and this stimulates a ventilatory response that overshoots, causing pCO2 to fall below threshold again.

125

Pain from an _______ is exacerbated by movements that cause stretching of the muscle, such as extension of the hip

Psoas major abscess- patients will typically position themselves with their hip flexed and lumbar lordosis

Psoas major muscle functions to extend the hips*

126

What DNA virus is partially double stranded, circular and enveloped?
Atypical lymphocytes found on blood smear and the virion is shown to possess an RNA dep-DNA pol activity

Hepatitis B- partially ds DNA to fully ds DNA to mRNA to ssDNA by its polymerase then to partially ds DNA again

Atypical lymphocytes are nonspecific symptoms of an infection* (EBV and CMV have high counts of these)

127

Diffusion across the alveolar membrane of O2 and CO2 is diffusion/perfusion limited (choose) normally

If the above answer is poor, equilibrium will occur slowly or not at all, resulting in what measurements of alveolar O2 and CO2

Perfusion limited

Tracheal air and alveolar air would have about the same pO2 values (around 150) and very low pCO2 levels

*in diffusion limited states, CO2 has much higher diffusion and is not nearly as affected as pO2 if there is diffusion limited problems

128

______ is the one non-glucose monosaccharide that can bypass ________, a key enzyme involved in regulating the rate of glycolysis, which is why it is metabolized faster than the other monosaccharides and is rapidly cleared from bloodstream
Metabolism of it in the liver consist of :

Fructose; bypass Phosphofructokinase

Phosphorylation by fructokinase to F-1-P. Aldolase B converts this to DHAP and glyceraldehyde (can be converted to G-3-P or DHAP) and enters the glycolysis pathway

129

purulent arthritis of the knee, oligoarticular joint pain, and vesiculopustular lesions on extremities indicates:

Disseminated gonoccocal infection due to Nisseria gonorrhoeae
*triad of polyarthralgia, dermatitis, and tenosynovitis

Septic arthritis by staph in young adults is usually linked to IV drug use and do not have dermatitis or polyarthralgia

130

Placing whole blood into a narrow tube and observing RBCs settling at the bottom, forming a rouleaux, is what test?
What cytokine stimulates production of the above?

ESR to identify acute phase reactants

IL-6 produces APR

131

Patient on an NE IV drip shows vein blanching and tissue surrounding the site to become cold, hard and pale- what is occurring?
What is the treatment?

NE extravasation from vein into the subcutaneous tissue causing overactivation of alpha 1 receptors, vasoconstriction and necrosis

Treatment- subcutaneous dose of alpha blocker (like phentolamine)

132

Fetal hydronephrosis- most common obstructive cause/site along the ureter and most common non-obstructive cause/site along ureter

Obstructive- ureteropelvic junction. Due to inadequate recanalization causing a narrowing
Non-obstructive- vesicoureteral reflux- from incomplete closure of this junction (ureter cannot be compressed enough to prevent backflow of Urine during bladder contraction)

133

Ventricular arrhythmias occurring shortly after an MI are typically treated with _______ class due to predominantly binding to rapidly depolarizing and ischemic ventricular myocardial fibers and has minimal effect on normal ventricular myocardium

Lidocaine
Class IB like lidocaine are weakest sodium channel blockers/dissociate the fastest and predominantly bind to sodium channels in the inactivated state and dissociation occurs so rapidly that they rarely have an effect on normal myocardium
Ischemic myocardium has higher than normal RMP, which delays recovery of sodium channels from the inactive state, which allows increased binding of class IB

134

Trendelenburg gait (hip drop as ipsilateral foot is lifted off the ground) is produced by damage to what nerve/muscle?
What quadrant is an injection placed in to cause damage here?

Contralateral superior gluteal nerve (L4-S1) or gluteus medius muscle

Injections in the superiomedial quadrant can cause this gait (nerve runs through the superior sciatic foramen above the piriformis)

135

Following IV administration of a highly lipophilic anesthetic, the drug will be _____distributed to organs first with _______, then soon after redistributed to organs with ______
Example of drug

Rapidly distributed going to highly vascularized organs first like the brain, kidney, liver and lung then redistributed quickly to poorly vascularized tissue like skeletal muscle, bone and fat

Propofol

136

Small cell lung cancer causing progressively worsening dizziness, limb and truncal ataxia, dysarthria, and visual disturbances from:
Pathogenesis?

Paraneoplastic cerebellar degeneration
Due to immune response against tumor cells that cross react with purkinjie neuron antigens causing rapid degeneration
*Anti-Yo, anti-P/Q, and anti-Hu antibodies in serum

137

What factors utilize the cAMP signaling pathway?

FSH/LH, ACTH, TSH, CRH, hCG, ADH V2, MSH, PTH/calcitonin, GHRH, glucagon, histamine H2

(Fat CAMP)

FSH/LH/hCG; ACTH/CRH/MSH; TSH, V2/H2, PTH/calcitonin; glucagon

138

G protein/inositol triphosphate (IP3) pathway activates ______ intracellular messenger that does what; end with increased intracellular _____ which leads to activation of _____
What utilizes this pathway?

G protein activation activates phospholipase C, which degrades phospholipids into IP3 and DAG. IP3 leads to increased intracellular calcium that leads to activation of protein kinase C with DAG

GnRH, oxytocin, ADH (V1), TRH, Histamine H1, AG II, Gastrin

139

What modulators utilize the cGMP intracellular signaling pathway

BNP, ANP, EDRF (NO)

140

Nonreceptor TK signaling pathway utilizes _____as a second messenger. This path is used by what ligands?

JAK/STAT pathway

Prolactin, GH, immunomodulators (cytokines, IL-2, 6 and IFN), G-CSF, erythopoietin, thrombopoietin

*think acidophils, cytokines, and blood components

141

Intracellular signaling pathway for how insulin activates glycogen synthesis
What other intracellular signaling pathway is present upon insulin binding and what does it result in?

What other modulators use TK signaling?

TK binding leads to phosphorylation of insulin receptor substrate (IRS-1), this activates several pathways: activation of phosphatidylinositol-3-kinase (PI3K) stimulates metabolic function like translocation of GLUT4 and glycogen synthesis by activating protein phosphatase, an enzyme that activates glycogen synthase by dephosphorylation

Another pathway activated is the MAP kinase pathway that leads to mitogenic functions such as DNA synthesis and cell growth

Others using TK: Growth factors! (Map kinase*) IGF-1, FGF, PDGF, EGF

142

_____ is a highly conserved DNA sequence that is usually about 180 nucleotides in length. A gene containing this sequence typically codes for ______

Homeobox
Typically codes for transcription factors that bind to regulatory regions on DNA, altering expression of genes involved in the segmental organization of the embryo: make sure structural elements of the body are formed in the correct position along the cranio-caudal axis

143

Cumulative incidence=

Total number of new cases/number of people at RISK at the beginning of the period. It does not account for deaths in the period under study. At risk population does not include those who already have the disease

144

_____ transduce signals from growth factor receptors such as epidermal growth factor that have intrinsic tyrosine kinase activity

ras proteins and MAP kinase

145

Child with blue-black pigment of the sclera, ear cartilage, nose/cheeks, arthralgias in adulthood and urine that turns black upon exposure to air
What is the disease?
What is the enzyme that is deficient?
What accumulates?

Alkaptonuria

Deficiency of homogentisate oxidase in the degradative pathway of tyrosine to fumarate; pigment forming homogentisic acid accumulates in tissue and urine
Auto recessive

146

all vitamins and minerals are present in breast milk in adequate amounts except _____ and ______

Vitamin D and K
Need vitamin D supplementation in exclusively breast fed infants. Vitamin K supplement is given intramuscularly at delivery

147

drugs metabolized by N-acetylation in the liver causing drug induced lupus
Who is at greater risk for developing DILE?

Procainamide, hydralazine, isoniazid

Slow acetylators

148

most specific diagnostic finding for pyelonephritis is:

WBC casts

149

MOA how Crohn’s forms gallstones and kidney stones, resp.

Gallstone- reduced bile acid reabsorption in terminal ileum causes an increased cholesterol:bile acid ratio, supersaturating the bile with cholesterol, leading to stones

Kidney- increased oxalate absorption (due to decreased calcium available for binding it in the gut) contributes to calcium oxalate stones

150

first branchial arch

Syndromes associated with defective first branchial arch formation?

Cartilage- maxillary process and mandibular process, malleus and incus, and sphenomandibular ligament
Muscles of mastication, mylohyoid, anterior belly of digastric, tensor tympani, anterior 2/3 of tongue, tensor veli palatini
Nerve- CN V3

Pierre robin sequence- micrognathia, glossoptosis, cleft palate, airway obstruction
Treacher Collins- neural crest dysfunction: mandibular hypoplasia, facial abnormalities

151

Aortic arch derivatives:
First-
Second-
Third-
Fourth-
Sixth-

Maxillary artery- branch of the external carotid
Stapedial artery and hyoid artery
Common carotid and proximal internal carotid
Left= aortic arch. Right= proximal right subclavian
Left= Proximal pulmonary arteries. Ductus arteriosus

152

where can the great saphenous vein be assessed? Route?

Superficial vein that courses along the medial side of the foot, courses anterior to the medial malleolus, up the medial aspect of the leg and thigh. Drains into the femoral vein in the femoral triangle, which is inferolateral to the pubic tubercle, where it can be assessed

153

insulin production begins with preproinsulin which begins with a N-terminal signal peptide that directs it to _____
Then it is cleaved and this form goes to the ______; where it is ____ and cleaved once more to make insulin

The RER to continue translation
Then the signal peptide is cleaved to make proinsulin and is sent to the Golgi to be packaged into secretory granules
Proinsulin is cleaved to insulin and C-peptide in secretory granules and are stored here until needed for release via exocytosis

154

Development of respiratory distress, confusion, and upper body petechial rash within days of severe long bone fractures:

Stains black with osmium tetroxide

Fat embolism syndrome in lung

155

Cancer treatment utilizing a dsRNA molecule that is complementary to a region of mRNA encoding pathogenic factors (like c-Myc in Burkitt’s) causing a significant reduction in this protein and in cell growth
What process does this treatment work through?

RNA interference- inhibits RNA translation via pottranscriptonal gene silencing
SiRNA and miRNA are examples - forms a ds RNA in the nucleus, exported to cytoplasm, cleaved into a short RNA helix by a protein called dicer*, these individual strands are incorporated into RNA-induced silencing complex where miRNA binds a complementary sequence on target mRNAs= mRNA degradation or translational repression

156

The overexpressed protein in Burkitt’s lymphoma has what activity?

C-Myc: nuclear phosphoprotein that functions as a transcription activator controlling cell proliferation, differentiation and apoptosis

157

Follow the path of fetal circulation
Which segment has the highest blood oxygen sat?

Oxygenated blood from the placenta is delivered to the fetus via the Umbilical vein- has 80% O2 saturation
Blood from the umbilical vein is first delivered to the liver, where it bypasses hepatic circulation via the ductus venosus* and enters the IVC—>heart—> mixes with deoxygenated blood from SVC—> pulmonary circulation (or through foramen ovale)—> bypass lungs via ductus arteriosus to descending aorta—>fetal tissue

Deoxygenated blood is delivered back to placenta by paired umbilical arteries

158

colorectal carcinomas from a history of UC are typically more _____, with a _____ grade, and evolve from ______ lesions, and is frequently multi/unifocal?
What mutation sequence underlies these carcinomas?

Colitis associated colorectal carcinomas are usually more aggressive, higher grade tumors.
They usually evolve from nonpolypoid/flat lesions, and are multifocal

Molecular pathogenesis is also different from sporadic disease in that it starts with mutation in p53 early and APC mutations occur later

159

a blueish neoplasm occurring under the nail bed- arises from ______, which is involved in what?

Modified smooth muscle cells of the thermoregulatory glomus body
Each glomus body is composed of an afferent arteriole, muscular AV anastomosis, and an efferent vein. Goal is to shunt blood away from the skin surface in cold temperature to prevent heat loss. Primarily in the pads of fingers, toes, dermis of nail bed and ears

160

Alpha-anti trypsin deficiency results in what type of lung disease?
What will PFTs look like?
Where does alpha-AT accumulate?

Obstructive- panacinar emphysema; lower lobes

Decreased FEV1/FVC ratio, increased TLC, decreased diffusing capacity for CO

Accumulates in ER of hepatocytes- PAS + globules

161

A CMV infection in an immunocompetent individual would likely present as ______

Mononucleosis like syndrome but with negative heterophile antibodies

162

The spinothalamic tract and dorsal columns mediate _____ information to the brain and synapse where in the brain?

What nucleus receives these modalities for the face?

A patient with one sided hemisensory loss likely suffered what type of stroke?

Pain, temperature, vibration, proprioception, light touch, pressure

Ventral postero-LATERAL nuclei of the thalamus

Facial sensation, pain and taste synapse in the ventral posterior-MEDIAL nucleus of the thalamus

A contralateral thalamic stroke* of the ventral posterior nucleus (a lesion to the posterior limb of the internal capsule would likely also affect the anterior IC, affecting motor signaling; rarely would cause a pure sensory)

163

Hemodynamic profile shows a difference between lower pressure aortic and higher LV pressure during systole, signifying ______ murmur. This murmur intensity is proportional to ______

Aortic stenosis

Intensity of the murmur is proportional to the pressure gradient between the LV and aorta during systole

164

______ is most appropriate next step in a patient presenting with a panic attack- chest pain, tachycardia, shortness of breath, and tremulousness and fear/feeling of dying in otherwise young healthy patient with normal ECG

Benzodiazepine administration

165

Patient with bleeding gums, cork-screw hairs, and low nutrient diet. The enzymes that are hypoactive are found in what organelle?

What disease is characterized by problems forming the triple helix of collagen?
What disease is characterized by inability to cleave disulfide rich terminal regions to make tropocollagen or crosslinking?

Patient has scurvy from Vit C def- enzymes that are hypoactive are in the RER*- hydroxylation step of lysine and proline takes place here and requires vitamin C

Osteogenesis imperfecta- occurs in RER, glycosylation of pro-alpha chain hydroxylysine residues and formation of procollagen via hydrogen and disulfide bonds to form triple helix

Ehlers-Danlos- these steps occurs in the extracellular matrix

166

Dephenoxylate is an ________ used as an antidiarrheal due to its MOA of:

Opioid agonist at the Mu receptor- slows motility

Close presynaptic calcium channels, open post synaptic potassium channels and decrease synaptic transmission to inhibit release of Ach, NE, serotonin, glutamate, substance P

167

Sepsis after surgery, patient found to have a central venous catheter, blood cultures and culture of the catheter tip on sheep blood agar grow elliptical, purple budding organisms. What is the likely organism?

Candida

168

_______ are likely to cause multiple spontaneous abortions or children born with severe anomalies (genetic term)

Unbalanced chromosome rearrangements

169

Antihistamines and ______ are used to treat allergic rhinitis

Alpha agonists- short term

170

Multiple history of malignancies seen at an early age from ______ mutation resulting in malignancy Li-Fraumeni syndrome: osteosarcoma, breast adenocarcinoma, leukemia and adrenal cortical gland tumors.

P53 mutation- impaired regulation of apoptosis
Blocks G1-S phase

171

Smoking causes what set of changes in respiratory defense function?
______ mucous production/secretion, ____ activity of airway cilia, and ______ alveolar macrophage function

Increased mucus, decreased activity of airway cilia, decreased alveolar macrophage function

172

Inactivation of GTPase of the Galpha subunit of G proteins would result in increased ______

Adenylyl cyclase

Guanylyl cyclase is broken down by PDE *

173

Patient with sickle cell disease presents with flank pain, low Hct, and UA shows gross blood and no WBC casts likely has

Renal papillary necrosis- GROSS blood and flank pain* esp in sickle cell patients, DM or severe pyelonephritis

174

In older men the most common pathogen causing prostatitis is ________

E. Coli

In younger men- chlamydia and gonorrhea

175

What 5 drugs are chosen during hypertensive emergencies?

Clevidipine, fendoldopam (D1 agonist vasodilates and increases natriuresis), labetalol, nicardipine or nitroprusside

176

Defect in _______ causes coarse facial features, gingival hyperplasia, clouded corneas, restricted joint movements, claw hand deformity, and a high plasma level of lysosomal enzymes

Defect in golgi’s ability to phosphorylate mannose residues (low mannose-6-phosphate on glycoproteins) causing proteins to be secreted extracellularly instead of into the lysosomes
I-cell disease*

177

Kidney _____ cells convert ________ to 1,25 dihydroxycholecalciferol via what enzyme?
What form is absorbed by the skin?
What form is produced by the liver?
What is the active form of vitamin D?

PCT cells convert 25-hydroxycholecalciferol via 1alphahydroxylase

Skin- cholecalciferol Vit D3
25 hydroxylase in liver produces 25-OHD3 to send to kidney to activate to the active form calcitriol

178

_________ occurs in areas of with repetitive sequences, where the DNA pol dissociated and upon reassociated, reattached at a position one or two positions ahead or behind on the repeat sequence. Underlies short tandem repeat mutation/microsatellites

Single strand mispairing

179

Which muscle is being tested on resisted empty can/thumbs down test of rotator cuff?

What are the other muscles of the rotator cuff and their actions/innervations?

Supraspinatus- abducts arm initially, Suprascapular nerve

Infraspinatus- externally rotates arm (pitching injury) suprascapular n
Teres minor- adducts and external rotates, axillary n
Subscapularis- adducts and internally rotates, upper and lower subscapular nerves

180

What organelle is affected if a patient shows signs accumulated VLFCA, phytanic acids, and pipecolic acid

Peroxisomes

*alpha oxidation is a strict peroxisome function that metabolizes phytanic acid to pristanic acid

181

Antibiotic therapy is likely to have what effect when given to a patient with salmonella?

Prolong fecal excretion of the organism (shortens for shigella)

Only to be given to patients with severe salmonella illness

182

Infant with micrognathia, glossoptosis, cleft palate and airway obstruction showing periauricular skin tags:
What pharyngeal arch is defective in this patient?
What normally makes up this arch

What other syndrome includes defects in this arch?

Pierre robin sequence- defective 1st pharyngeal arch

Maxillary process, mandibular process, malleus and incus, muscles of masstication, tensor tympani, anterior 2/3 of tongue, tensor veli palatini

Treacher-Collins syndrome- neural crest dysfunction- mandibular hypoplasia and facial abnormalities

183

Patient with long standing type 2 DM treated with gemfibrozil comes in with a complaint of pain in his feet, more severe at night and is relieved by taking a hot bath. Achilles DTR is diminished and sensation to pinprick and vibration is decreased from just above the ankle distally. How would the patient describe the pain? (One word)

Burning

184

drug against nematodes and cestodes that increases the permeability of the cell membranes to calcium causing paralysis, dislodgment and death of the parasite

Praziquantel

185

Which of the bone structures assists in producing new bone to heal fractures?

Periosteum.

186

Presents with signs of fat malabs (vitamin deficiency and steatorrhea), failure to thrive, retinitis pigmentosa, spinocerebellar degeneration due to vitamin E deficiency, progressive ataxia and acanthocytes

Abetalipoproteinemia- deficiency of ApoB48 and B100
Auto recessive disease. Chlyomicrons, VLDL and LDL are absent

187

_______ is also known as osteoclasts activating factor

IL-1- increased in mechanisms that show lytic bone lesions

188

Infant 46, XX girl with ambiguous external genitalia and clitoromegaly with elevated levels of testosterone and androstenedione. During pregnancy, the mother experienced facial hair growth and voice deepening. What enzyme was likely deficient?

Aromatase deficiency

21alpha hydroxylase CAH is most common cause of this presentation in newborns, but does not cause mother virilization

189

Which type of porphyria causes photosensitivity?
Which type causes abdominal pain, port wine colored urine, neuropsychiatric disturbances and is usually precipitated by cytP450 inducers?

Porphyria cutanea tarda- deficiency of uroporphyrinogen decarboxylase

Acute intermittent porphyria - earlier step of porphyrin synthesis is affected. Porphobilinogen deaminase deficiency

190

Bacteria suspended in isotonic solution displays a spherical configuration and then disintegrates rapidly when placed in a hypotonic solution- what aspect of the bacteria is likely damaged from this observation?

Bacterial cell wall of Gram + bacteria- usually resists damage by osmotic forces/changes in tonicity

191

Common causes of myopathy with increased CK include

Elevated CK myopathy, muscle cramps and proximal weakness, delayed tendon reflexes with weight gain, bradycardia and brittle nails/splitting of nails is likely which of the above?

Hypothyroidism, muscular dystrophy, inflammatory muscle disease, and statins

Hypothyroid myopathy

192

Patient with acute pancreatitis with normal gallbladder and no alcohol use- what lab would you check and why?

Serum TGs, if over 1000, FFA conc likely exceeds the binding capacity of albumin and leads to direct injury to pancreatic acinar cells

193

Infant presents with 4 days of high fevers that commonly elicit seizures followed days later by a maculopapular rash on trunk- what virus?

Roseola- HHV6

Compare to measles- rubeola paramyxoviridae that presents with 3C’s, then koplik spots, then maculopapular rash that STARTS on the face and spread downward (roseola rash starts on the trunk and spreads to face)

194

What structure is affected by transtentorial herniations?
What structure is affected by subfalcine herniations?
What structure is affected by tonsillar herniations?

Oculomotor nerve and PCA (uncal herniation from the medial temporal lobe herniations through the gap between the crus cerebri and the tentorium)

ACA is compressed- cingulate gurus herniated under falx cerebri

Medulla is compressed- cerebellar tonsil herniated through foramen magnum

195

What birth defect is associated with prenatal screening finding of decreased AFP or increased unchallenged translucency on US?

Down syndrome

196

Calculating RBF=

Renal plasma flow divided by 1-Hct

Renal plasma flow= PAH clearance= urine [PAH]xurine flow rate/ plasma [PAH]

RBF= PAH clearance/1-Hct (1-Hct)=plasma volume

197

Malignant tumor arising from _______ and is most commonly preceded by a pregnancy and weeks later causes vaginal bleeding, uterine enlargement and significantly increased beta hCG
Early spread to other organs via ______; most commonly to what organ?

Gestational choriocarcinoma- malignant proliferation of trophoblasts

Rapidly invades the uterine wall and undergoes hematogenous spread- most commonly to the lungs (“cannonball” metastasis)

198

Pro-carcinogens are converted to active metabolites by _____

Microsomal cyt P450 monoxygenase

199

Gastric varices can be seen in portal HTN and _____ due to chronic pancreatitis. What veins drain into this vein?

Splenic vein thrombosis - this vein runs along the posterior surface of the pancreas and can develop a blood clot from pancreatic inflammation

Short gastric veins drain the fundus of the stomach into the splenic vein, so these can experience increased pressure and cause gastric varices in the fundus

200

Young patient with signs of acute coronary syndrome and MI and increased methionine levels likely has ________ deficiency
What becomes essential?
Other associated findings with this disease?

Cystathionine synthase deficiency resulting in homocystinuria and shunting of the pathway to methionine synthesis

Cysteine becomes an essential AA- and vitamin B6

Osteoporosis, marfanoid habitus, ocular changes (down and in lens subluxation), CV events, and kyphosis

201

Patient with bipolar disorder experiencing tremor, ataxia, and involuntary movements started a new BP medication weeks ago. What anti-hypertensive is most likely being used?

Thiazides, ACE- anything that decreases volume or GFR increases lithium toxicity (chronic presents at neurological deficits)
Lithium gets reabs with sodium in the PCT- decreased volume will cause later increased reabs of sodium and water in PCT and therefore increased lithium

202

Amatoxins in mushrooms inhibit ______ predominantly in what organ?

mRNA synthesis (inhibiting DNA dep RNA pol II) typically in liver cells- taken to the liver from gut via portal vein

203

what are the 3 stop codons?

UAA, UAG, UGA

204

Toxicity/overdose of insecticides/pesticides contains _____, which gives a garlic odor to the breath, copious watery diarrhea, and QT prolongation, binds to _______; impairing cell respiration and GNG by inhibiting pyruvate DH
What is the treatment?

Arsenic poisoning; binds sulfhydryl groups and inhibits lipoic acid

Dimercaprol- has a sulfhydryl group to combine with arsenic

205

Combination of hemolytic anemia (antibodies to CD55 and CD59 found on flow cytometry), hypercoagulability/thrombosis and pancytopenia indicates ________, which is a mutation in the ______ gene that synthesizes _______

Paroxysmal nocturnal hemoglobinuria- defect in the PIGA gene that usually synthesizes GIP anchor protein that holds decay accelerating factor and MAC inhibitory protein to the RBC membrane in order to inhibit complement activation

Without this anchor factor, CD55/DAF and CD59/MIP are absent and there is complement mediated lysis of RBC, WBC and platelets

Hemolysis occurs more often at night due to the respiratory acidosis that occurs during sleep pattern breathing

Thrombosis occurs due to lysis of platelets releasing prothrombotic factors and free Hb from RBCs

206

Patient has diplopia when walking down stairs or reading things up close, eye shows hypertropia and extortion- what nerve is affected? What is the normal function of the muscle(s) it innervates?

Trochlear nerve- superior oblique muscle- usually intorsion upon abduction and downward gaze upon adduction
Lesion is vertical deviation and extorted

Compensation- chin is tucked and head is tilted away from the affected eye to compensate for hypertropia and extortion

207

Chronic allograft rejection would show
Acute allograft rejection would show

Obliterating intimal thickening, tubular atrophy, and interstitial fibrosis (shrunken organ; arteriolosclerosis)

Interstitial lymphocytic infiltrates

208

Infant with neurological deficits and increased serum lactate levels has what primary enzymatic deficiency? What AA/diet treats this condition?

Pyruvate dehydrogenase deficiency- causes buildup of pyruvate that gets shunts to lactate via LDH and alanine via ALT.
X-linked

Treatment is a ketogenic diet- high fat and ketogenic AA like lysine and leucine, which are broken down to acetyl-CoA and cannot be metabolized to pyruvate or glucose.

209

What kind of murmur does a VSD have?
What would happen to the SpO2 in the LV?

Holosystolic, harsh sounding murmur best heard over the lower left sternal border.

Stays relatively normal with smaller VSDs; larger VSDs allow equalization of chamber pressures

210

Pulmonary lesion culture grows Fusobacterium and Peptostreptococcus- indicative of?

Anaerobic bacteria that are a part of the normal flora in mouth
Highly suggestive of lung abscess with foul smelling sputum- aspiration is most common cause

Also bacteriodes and prevotella

211

Thiazides increase Ca reabsorption from tubules through 2 major mechanisms:
Indicated in patients with _____

Blocking Na/CL cotransporter on the apical side decreases intracellular Na concentration, which stimulates the basolateral Na/Ca anti-porter, pumping Na into the cell and reabsorbing calcium into the blood from the tubules.

Hypovolemia increases Na and H20 reabsorption in the PCT, which increases passive paracellular Ca reabs

Nephrolithiasis secondary due to hypercalciuria

212

Impaired ________ causes hypoglycemia after prolonged periods of fasting and inappropriately low levels of ketone bodies. _______ enzyme deficiency is the most common cause and catalyze the first step of this process

Fatty acid beta-oxidation

Defect in Acyl-CoA dehydrogenase

213

X linked recessive deficit of CD40L and CD40 interaction causes _______, presenting with recurrent sinopulmonary and GI infections, opportunistic pathogens, and failure to thrive in children.

Normally, what allows for this missing process to occur?

Hyperimmunoglobulin M syndrome - defective signaling between CD4+ T cells and B cells, which results in a defect of class switching

Isotype switching occurs by splicing out DNA coding for different heavy chain constant regions. The variable region/antigenic specificity of the AB stays the same.

214

Breast pathology that is associated with ‘orange peel’ skin rash- erythematous, itchy, firm and coarse. Significant edema, lymphadenopathy, but no mass

Inflammatory breast cancer- cancerous cells obstructing lymphatic drainage after spread to dermal lymphatic spaces

*can easily be mistaken for mastitis- but note no fever and longer time frame of rash than an infection

215

Prevention of neonatal tetanus focuses on ________

Vaccinating PREGNANT women or those who may become pregnant- pass IgG trans placentally to the fetus

216

The carpal tunnel contains the flexor digitorum _____, _______ and the ____ nerve. It is covered by the _________, which attaches to the hamate and pisiform on the ulnar side and the scaphoid and trapezium on radial side

Flexor digitorum profundus tendons and superficialis tendons, flexor pollicis longus, and the median nerve
Covered by the transverse carpal ligament (flexor retinaculum)

217

CAAT box and TATA box (promoter regions) promote initiation of transcription by acting as binding sites for _____ and _____

Transcription factors and RNA pol II

218

Characteristic histologic findings of GERD/Gastroesophageal junction incompetence

Basal Zone hyperplasia
Lamina propria papillae
Scattered eosinophils and neutrophils

219

Hematogenous Osteomyelitis in children usually affects what part of long bones?

Metaphysis - contains slow flowing, sinusoidal vasculature that is conducive to microbial passage

220

Lipid lowering agent that decreases hepatic synthesis of TGs and VLDL and reduces clearance of HDL. Most effective at raising HDL, but does not ________ so is typically only used in those who have failed other lipid lowering agents.
AE?

Niacin- does not reduce the risk of cardiac events

Decreases the renal excretion of uric acid- acute gouty arthritis
Hepatoxicity, hyperglycemia and flushed faces

221

Flat, greasy, purple/brown pigmented ‘stuck on’ appearance lesions- histologically would show what?
Rapid onset of multiple of these lesions would be associated with what?

Seborrheic keratosis- show keratin filled cysts, hyperkeratosis (proliferation of stratum corneum)

Underlying GI or lymphoid malignancy- Leser-Trelat sign

222

DoC in a patient with DM and HTN to prevent chronic kidney disease

ACE-I and ARBS (if intolerant to bradykinin/substance P increase causing cough)

223

Early course of DM II is characterized by increased ____, but later course shows resistance and decreased levels. At this point, what will be elevated in the blood that further contributes to the disease? (Besides glucose)

Insulin production increases

Insulin resistance stage- show elevated FFA levels (insulin usually have antilipolytic effects) and further contributes to insulin resistance by impairing insulin dependent glucose uptake and increasing GNG in liver

224

Corneal reflex involves ____ afferent limb and _____ efferent limb

What nerve innervates the levator palpebrae muscle (when paralyzes, creates ptosis)

CN V1 and CN VII

CN III

225

Ether and other organic solvents dissolve ______ viruses

Enveloped- causing them to lose their infectivity

226

A conscious choice not to dwell on a particular thought or feeling=

Making excuses for unacceptable feelings/actions, usually to avoid self blame=

Involuntarily withholding an idea or feeling from conscious awareness=

Suppression- mature defense

Rationalization- immature defense

Repression- immature defense

227

Gardos Ca channel blockers hinder ________ from the cell, preventing _____ of erythrocytes and reducing polymerization of HbS

Efflux of potassium and water from the cell, preventing dehydration of RBCs in sickle cell patients

228

NF 1 is on chromosome _____ ; which codes for what protein and presents with ______
NF2 is on chromosome _____; which codes for what protein and presents with _______

NF1 is on chromosome 17, which codes for neurofibromin (Ras GTPase activating protein), and presents with cafe-au-last spots, multiple neurofibromas, lisch nodules (gold/tan domes on surface of iris)

NF2 is on chromosome 22, which codes for Merlin (schwannomin) and presents with bilateral acoustic neuromas (remember: 2–22-2 neuromas (proliferation of myelin))

229

Complement binds to the Fc portion of IgM and IgG closer/further from the hinge region?
Which portion does the antigen attach to?

Closer* more proximal end of Fc (near the disulfide bonds). The distal end of Fc portion serves at the site of attachment to the phagocytic cells

Antigen attaches to the Fab region (variable region) of the light chain and heavy chain

230

What type of collagen is seen in scar formation? What other structures contain this type of collagen?

What type of collagen is seen in granulation tissue?

Type I in scar formation- as seen 2 weeks+ after an MI
Type I is also seen in bone,tendons, ligaments, dermis, cornea and BVs

Type III collagen in granulation tissue

231

During transient ischemic attacks on myocardium, anaerobic metabolism takes over, which creates a decrease in production of ______, which leads to _____ and _____ accumulation in the cell

Which accumulation leads to failure of further myocardium contraction?

ATP—> Na and Ca accumulation (Na/K pump and SR Ca ATPases fail)- this then causes water to flow into the cell and swell (increase in cell size)

*if sarcoplasmic reticulum cannot sequester Ca, it will lead to a cessation of contraction within the ischemic zones of myocardium

232

Formation of atheroma: endothelial cell injury leads to increased expression of surface _____ to allow adherence and migration of monocytes
Once there, they release cytokines and GFs that promote migration and proliferation of _____, which are stimulated to synthesize extracellular matrix proteins and formation of the _______

Vascular cell adhesion molecules
Vascular smooth muscle cells
Fibrous cap

233

Pituitary adenoma: ACTH and cortisol is/not suppressed by high dose dexamethasone test
Ectopic ACTH production (lung cancer: ACTH and cortisol is/not suppressed by high dose dexamethasone test

Pituitary adenoma IS suppressed by high dose

Ectopic is NOT suppressed by even high doses of dexamethasone

Think of ectopic ACTH as being non responsive to central feedback since it is not apart of the normal CNS pathway

234

As blood conc of PAH increases, the extraction rate decreases progressively due to effects on (filtration/secretion: choose), why?

Secretion; carrier mediated transport is saturated as PAH conc increases. There is a Max secretion rate*
PAH is constantly filtered no matter the blood conc, so this is not affected and cannot be saturated.

235

________ carcinoma is characterized by overlapping nuclei containing finely dispersed chromatin, intranuclear inclusions and grooves due to invagination of nuclear membrane, and psammoma bodies (calcium deposits)

Papillary carcinoma of thyroid- overlapping nuclei with finely dispersed chromatin give the cells an empty or ground glass appearance (orphan Annie nuclei)


Tumors with psammoma bodies- PSaMMoma: papillary thyroid, serous cystadenocarcinoma of ovary, mesothelioma, and meningioma

236

Traumatic aortic rupture caused by a rapid deceleration (MVC) most commonly injures ______ site on the aorta

Aortic isthmus, which is tethered by the ligamentum arteriosum (right after the brachiocephalic, common carotid, and subclavian artery branch off)

237

treat restless leg syndrome with _____ (mechanism of drug)
Most common cause of RLS is _____

Dopamine agonists (ropinirole, pramipexole)

Iron deficiency or diabetic neuropathy in older adults

238

History of calculous cholecystitis is now found to have air in the gallbladder and biliary tree. Where did the stone most likely end up?

Perforation of the gallbladder wall usually into the duodenum via a fistula will cause gas to be present in the gallbladder; the stone will move unimpeded until hitting the ileum, causing a gallstone ileus** and subsequent small bowel obstruction.

239

Enteric bacteria produce _____ and _____
SIBO is a risk factor in _____ surgery
SIBO results in a deficiency of what vitamins and minerals?

Vitamin K and folate- overproduced in SIBO

Increased risk in gastric bypass surgery- bloating, nausea, abdominal discomfort, and malabsorption

Deficiency of vitamins B12, A, D, and E and iron.

240

Pregnant mother with a history of irregular menses that last 3-4 days undergoes a quadruple screening test that shows decreased AFP. What is the likely etiology of the low AFP?

Dating error- women with irregular menses are at risk for inaccurate pregnancy dating, which is vital for an accurate quadruple screening test

If correct, a low AFP indicates aneuploidies like trisomy 18 and 21

241

Which step involved in processing and handling of mRNA occurs only within the cytoplasm?

Interaction with P bodies- that hold micro-RNA induced mRNA silencing control, exonucleases and recapping enzymes for translation repression and mRNA decay.

Posttranslational modifications like 5’ capping, poly A tail, and intron splicing occur before leaving the nucleus

242

Indolent lymphoma that is often presenting with a painless ‘waxing and waning’ lymphadenopathy over years
Translocation?

Follicular lymphoma
T(14;18)- BCL-2 inhibits apoptosis by keeping the mitochondrial membrane impermeable, preventing cytochrome C release to decrease caspase activation

243

Newborn born of a natural birth presents with a large, bulging anterior fontanelle, eyes driven downward and unable to track upward, and intracranial hemorrhage in confirmed on CT. What is the most likely cause?

Vitamin K deficiency

If this patient had received vitamin K after birth, then abusive head trauma would be the likely culprit causing shaken baby syndrome

244

Bleeding due to heparin is reversed immediately by administration of:

Protamine sulfate- peptide that binds heparin, forming a complex that has no anticoagulant activity
*FFP is not useful for heparin overdose as it contains ATIII, which can further potentiate the effects of heparin

245

Infant born evaluated for hearing loss, white pupils and a continuous machine-like murmur:
Interventions that could have prevented this:

Congenital Rubella (Togavirus)- cataracts, sensory-neural deafness, and PDA

Live attenuated rubella vaccine to the mother before conception

246

Patient comes in with hypertensive emergency (BP over 180/120) and evidence of end organ damage (encephalopathy, pulmonary edema, AKI, SAH, dissection, papilledema, etc). What medication is best- vasodilates arteries, improves renal perfusion and increases naturiesis? MOA?

Fenoldopam IV- short acting, selective, peripheral dopamine-1 receptor agonist, with little to no effect on alpha or beta receptors. Stimulation at this receptor activates AC and increases cAMP, resulting in vasodilation of arteries, renal vasodilation, and naturiesis.

247

Which level of the respiratory system holds the least airway resistance?
Cartilage and goblet cells extend to ______
Cilia terminate in the ______

Terminal bronchioles (ciliated simple cuboidal epithelium)

End of the bronchi

Respiratory bronchioles

248

Post surgical acute hepatitis could be from drug administration of:
It causes a _____ necrosis that is indistinguishable from viral hepatitis

Halothane- metabolized by hepatic cytochrome P450 system, converted to reactive intermediates that can directly injure the liver or lead to immune mediated hepatocellular damage

Centrilobular hepatic necrosis

Newer halogenated anesthetics have less liver toxicity- desflurane, sevoflurane, enflurane, isoflurane.

249

Which two heart murmurs decrease in intensity with squatting? (Most increase)
Primary _____ (type of murmur above) is a sporadic disorder often due to myxomatous degeneration, which is degeneration of _______.

MVP and HCM -due to increased LV volume

Primary MVP is due to degeneration of CT affecting the mitral valve leaflets and chordae tendinae (secondary MVP is due to CT disorders like marfans and Ehlers-Danlos

250

Arterial PaCO2 is a direct indicator of alveolar ventilation status. Hypocapnia implies ongoing _______
Upper airway obstruction, reduced ventilator drive, respiratory muscle fatigue, decreased chest wall compliance causes alveolar ______ and hypercapnia

Hyperventilation- from a V/Q mismatch that causes decreased O2 and CO2 exchange. Hypoxemia stimulates peripheral chemoreceptors and increases the respiratory drive

Hypoventilation

251

Patient with diabetic ketoacidosis now has facial pain, headache and nasal black eschar
Confirm diagnosis by?

Mucormyocosis- causing rhinocerebral infection acquired by inhalation of spores, ascends to orbits and penetrates the cribiform plate into the brain
Diagnosis is made by mucosal biopsy- broad, ribbon like nonseptate hyphae with right angle branching. Tissue invasion along BVs,

252

Patient with choking spells, dysphagia and cough, recurrent pneumonia, with foul smelling breath. Abnormality is found on barium swallow in upper esophagus demonstrating a :
Caused by?

Zenker diverticulitum caused by cricopharyngeal motor dysfunction
Motor dismotility causes herniation of mucosal tissue between the thyropharyngeal and cricopharyngeal parts of the inferior pharyngeal constrictor

Bad breath- food stuck in diverticulum
Pneumonia- recurrent aspiration of food bolus

253

The defective receptors in familial hypocalciuric hypercalcemia are what type of receptors?

Calcium sensing receptors- transmembrane G protein coupled receptors that help regulate the secretion of PTH. Binding of this receptor leads to inhibition of PTH

Defective receptor in the PTH gland and the kidney

254

Reactive arthritis is commonly associated with _____; a ______ spondylarthropathy
Extra findings outside of classic triad of conjunctivitis, urethritis and arthritis

HLAb27- a seronegative spondylarthropathy

Skin findings- keratoderma blennorhagicum (hyerpkeratotic vesicles on palms and soles) and circinate balanitis (serpiginous annular dermatitis of glans penis). Axial involvement- sacroiliitis

255

Glucose uptake undergoes ______ transport across cell membranes

Facilitated diffusion- carrier mediated transport via GLUT receptors
Does not require energy- high to low conc across a transport protein

256

The _______ structure represents the most commonly identified DNA binding domain in humans which many transcription factors use to recognize specific genes and alter their activity
Which type of cell receptor can act like this?

Zinc-finger structure- chains of AA bound together around a zinc atom via linkages with cysteine and histidine residues, forming a stable structure of 2 antiparallel beta strands and an alpha helix

Intracellular receptors that act directly as TFs- lipid soluble hormones are the ligand

257

_______ is the most effective modifiable risk factor for preventing falls in the elderly

Medication review (increased risk of falls: psychotropic, CV, NSAIDs

258

_______ deficiency results in a symptomatic intolerance after juice, honey, or fruit consumption that presents with hypoglycemia, vomiting, jaundice, and cirrhosis
What substrate accumulates?

Aldolase B deficiency- hereditary fructose intolerance

Fructose-1-phosphate accumulates/trapped in cells, depletes body of phosphate, which results in inhibition of glycogenolysis and gluconeogenesis.

259

Excessive consumption of dietary ______ can produce Cytosine deamination, which creates _______
What is the sequence of repair? _____ recognizes and cleaves the altered base, ______ cleaves the 5’ end and _____ cleaves the 3’ sugar phosphate end, _____ fills the single nucleotide gap, and ____ seals it back up

Consumption of nitrites; Unusual uracil base

Glycosylase- leaving an AP site
Endonuclease
Lyase
DNA pol
Ligase

260

pathogenesis of atherosclerosis most likely begins with ______
This leads to ____ migration and exposure of _______ to allow _____ adhesion.
Eventual ______ migration into the intima and increased vascular permeability allow _______ into the intima

Endothelial cell injury - this dysfunction then leads to monocytes and lymphocyte adhesion and migration into the intima while exposure of subendothelial collagen promotes platelet adhesion. Growth factors produces by monocytes and platelets stimulate medial smooth muscle cell migration into intima. Increased vascular permeability allows LDL cholesterol into the intima, to be phagocytosed by macrophages.

261

During cardiac catch, puncture above the inguinal ligament can lead to a hemorrhage where?
Controlled with external compression?

Retroperitoneal hemorrhage- this portion of the femoral artery lies directly inferior to the peritoneum

Cannot be controlled with external compression and leads to hemodynamics instability

262

Two symptom categories in hypoglycemia are:
Beta blockers would affect which of these symptoms during hypoglycemia?

Neurogenic symptoms- post ganglionic sympathetic response mediated by NE/Epi and Ach. NE causes tremulouseness, palpitations, anxiety. Ach causes sweating, hunger and paresthesias

Neuroglycopenic- behavioral changes, confusion, visual disturbances, seizures

Nonselective Beta blockers (propranolol, timolol) block the NE component of the symptoms- so a patient would only experience the Ach portion- sweating, hunger and paresthesias

263

Rapidly progressive dementia and myoclonic jerks and ataxia
Brain biopsy showed gray matter changes with many uniform vacuoles between neuronal cell bodies and in the perikaryon

Creutzfeldt-Jakob disease

264

Child with irritability, vomiting, developmental delay, and large head circumference.
CT shows large empty posterior fossa

Dandy-walker malformation- absence of cerebellar vermis and cystic dilation of the fourth ventricle with posterior fossa enlargement

265

Raised, homogenous pigmented lesion with regular borders. Biopsy showed nests of uniform round cells at the basal portion of the epidermis that extend into the underlying dermis as well

Compound nevus- has both epidermal and dermal nests of cells

Junctional- flat lesion, has nests of cells at the epidermal-dermal border
Intradermal- papillary lesion, no pigment, only dermal nests of cells

266

Warfarin induced skin necrosis occurs in _______ deficiency

Individuals with a protein C or S deficiency- the initial transient hypercoaguable state after starting warfarin is exaggerated, promoting microvasculature occlusion and hemorrhagic skin necrosis

267

What are the strongest risk factors for Hep B transmission during delivery?

Maternal viral load and HBeAg

Infants will have high replication rate and HBeAg despite being asymptomatic with normal liver function testing

268

Proximal ureter is supplied by ______ artery
Distal ureteric is supplied by ______ artery

Renal artery
Superior vesicle artery

Inbetween- anastomoses can be highly variable coming from the gonadal, common and internal iliac, aorta or uterine artery

269

Differentiating bipolar I and II; the mania in bipolar II is characterized by a noticeable change in behavior but are often lacking _______ and ________

They both have:

Lack a functional impairment and psychosis needing hospitalization
Typically a change in behavior is noticed, but are still able to work efficiently without as much sleep.

Both bipolar I and II have depressive episodes

270

For PCR, the DNA template that includes the target region to be amplified and _______ are required for replication by a thermos table DNA polymerase with a pool of deoxynucleotide triphosphates

The flanking sequences adjacent to the target region- must be known in order to make primers necessary to start PCR

(The exact nucleotide sequence of the target region does not need to be known)**

271

What enzymes are affected in lead poisoning?
What substrates are accumulated?
Why is basophilic stippling observed?

Presentation in kids vs. adults

ALA dehydratase and ferrochelatase (protoporphyrin cannot combine with iron to form heme in the mito, incorporates a zinc ion instead)

ALA and zinc-protoporphyrin accumulated

Also inhibits rRNA degradation, accumulates in RBCs and stained. Microcytic anemia

Kids- mental deterioration due to incomplete BBB, abdominal pain. Have to use succimer for chelation in kids.
Adults- HA, memory loss, demyelination (wrist or foot drop). Dimercaprol and EDTA are first line treatment

272

In a patient with a total gastrectomy, what will they need a lifelong supply of?

Vitamin B12 even without IF

Protein digestion can still occur with pancreatic and intestinal protease

273

_____ is the step of leukocyte adhesion where increased vascular leakage in the vasculature leads to hemoconcentration and decreased wall shear stress, improving contact of neutrophils with endothelial lining

_______ occurs through binding of CD18 beta 2 integrins (Mac-1 and LFA-1) to ICAM-1 on endothelial cells


Transmigration is accomplished through integrin attachment and adherence to ______

Margination- step 1

Tight adhesion

PECAM-1- platelet endothelial cell adhesion molecule-1

274

What is the most common CFTR mutation in CF?
What drug can partially correct this defect?

3 base pair deletion of Phe at the 508 position (F508 change)
This mutation causes impaired post transactional modification processing- improper folding and glycosylation of CFTR

This abnormality is sensed by the ER and is targeted for proteasomal degradation, preventing it from reaching the cell surface.

Lumacaftor

275

Neural crest derived structures MOTEL PASS:

Melanocytes, odontoblasts, tracheal cartilage, enterochromaffin cells, laryngeal cartilage, parafollicular cells of thyroid, adrenal medulla and ALL ganglia, Schwann cells and spiral membrane

276

intestinal type gastric adenocarcinoma forms a _______ and is composed of ______
Diffuse carcinoma involves ________ due to loss of ______and display ______ on LM

Solid mass that projects into the stomach lumen that often ulcerates and is composed of glandular forming cuboidal or columnar cells
Diffuse carcinoma infiltrates the stomach wall diffusely due to loss of E-Catherine and display mucin droplets that push nucleus to the side forming signet ring cells

277

what is the most important poor prognostic factor in post-strep GN?

Increased age, only 60% of adult cases completely resolve
Can develop chronic HTN, recurrent proteinuria, chronic renal insufficiency and RPGN

278

Excess folate and recycled DHF are stored within cells via
________. So one would find this form increased while taking what drug?

Polyglutamation

Methotrexate use would cause an increase in dihydrofolate polyglutamate due to folate accumulate

279

Marcus Gunn pupil (afferent pupillary defect) is often seen in ispilateral optic nerve lesions, like ______ and _____. But also can be seen in contralateral ______ lesions causing contralateral homonymous hemianopsia, because:

Optic neuritis and MS

Also in optic tract lesions -since the nasal portion of the retina contributes more input to the pretectal nucleus (involved in afferent pupillary light reflex) than the temporal portion of the retina

280

Patient with hypertriglyceridemia and history of gallbladder disease/stones, what lipid lowering agent should be avoided and why?

Fibrates* (gemfibrozil for Gallstones)

Unregulated LPL- increased ox of FA. And inhibit the rate limiting enzyme in bile acid synthesis, cholesterol 7-alpha hydroxylase. Reduced bile acid production results in decreased cholesterol solubility, promoting stones.

281

gram positive cocci involved in endocarditis that can produce dextrans using sucrose

Strep viridans (S. Mutans and S. Sanguinis)

282

HIV associated dementia typically affects _____ structures in the brain, causing attention/working memory problems, executive dysfunction, and slow info processing.
Histopathological findings would shows _____

Subcortical and deep gray matter structures

Inflammatory activation of microglial cells forming groups/nodules

283

effective add-on therapy for severe asthmatics to reduce frequency of exacerbations and dependency on steroids?

MAB against IgE- omalizumab

284

Patient with history of migraines (takes amitriptyline), insomnia (takes diphenhydramine), and statin and diclofenac use is experiencing anticholinergic effects. Which medication should be stopped?

Diphenhydramine- higher anticholinergic affects
SE usually seen when combined with another anticholinergic- like amitriptyline

285

Risk factors for calcium oxalate stone:
_____ calcium in urine; diet
_____ oxalate in urine; diet
______ citrate in urine

Increased calcium in urine; decreased calcium in diet (increased calcium in diet binds oxalate in gut and won’t be absorbed) (decreased in diet can cause hyperoxaluria since less calcium is available to bind and trap oxalate)

High oxalate in urine (from Crohn’s); high oxalate in diet

Low citrate in urine- potassium citrate is usually the treatment for these stones to prevent their formation

286

Major consequence of scarcity of transverse tubules in skeletal muscle causing weakness is by:

Uncoordinated muscle contraction

T-tubules allow depol impulses to propagate rapidly through the interior of the muscle fiber, ensuring calcium release from SR occurs in a uniform manner to allow for syncronized contraction of myofibrils in each muscle cell.

287

Which CN exits the brainstem at the lateral aspect of the mid-pons at the level of the middle cerebellar peduncles
What would a stroke in this area look like?

Trigeminal nerve

Involving the anterior pons would affect the corticospinal tract (contralateral hemiparesis, Babinski sign) and. Corticobulbar tract (contralateral lower facial palsy, dysarthria)
Disruption of corticopontine fibers that convey motor info to the ipsilateral pontine gray matter results in contralateral dysmetria and dysdiadochokinesia (ataxis hemiparesis)

*pontocerebellar fibers arising from the pontine gray matter decussate and enter the cerebellum through the contralateral middle cerebellar peduncle

288

What enzyme cofactors is likely deficient in a patient with combined hyperphenylalanemia and elevated prolactin with axial hypotonia and microcephaly?

Deficiency of dihydrobiopterin reductase-takes BH2–>BH4

BH4 is used in the phenylalanine hydroxylase reaction to make tyrosine and also in the tyrosine hydroxylase reaction which makes DOPA
Less DOPA production= prolactin freed from inhibition

PKU is the phenylalanine hydroxylase deficiency, so supplement with tyrosine. But if the cofactor is deficient, then both reactions cannot take place.

289

Patient presents with progressive development of spastic diplegia, abnormal movements, cognitive and growth delay in the presence of high arginine levels in the plasma and CSF. What enzyme is deficient? What does this enzyme usually help produce?
Unlike other disorders of this class, what special characteristic does it have on lab exam?

Arginase deficiency, which is a urea cycle enzyme that produces urea and ornithine from arginine

Will have mild or no hyperammonemia*

Treat- low protein diet devoid of arginine and admin of synthetic protein with essential AA

290

TNF alpha and beta are synthesized in response to viral infections, resulting in transcription of antiviral enzymes that _________
These enzymes becomes active only in the presence of:

Halt protein synthesis

Ds RNA, which forms as a result of viral replication in infected cells- selectively affects infected cells protein synthesis*

291

Most gut tissue is ________ embryologic origin, but the _______ is mesoderm

Endoderm; spleen

292

Best approach to somatic symptom disorder?

Schedule regular visits with PCP, who can monitor and avoid unnecessary diagnostic testing and specialist referrals

293

Two important acid buffers in renal tubules are ________

NH3 and HPO4 2- ——-> NH4+ and H2PO4-

H2PO4- is increased in metabolic acidosis from accepting protons

294

What type of bias occurs in prospective studies, if loss of follow up occurs disproportionately between the exposed and unexposed groups?

What type of bias occurs when a screening test diagnoses a disease earlier than it would have appeared by natural history alone, so that the time of diagnosis until death appears prolonged even though there might not actually be any significant improvement in survival?

Attrition bias (a type of selection bias)

Lead-time bias

295

A _______ aneurysm is associated with acute/chronic hypertensive intracerebral hemorrhage, typically seen in the basal ganglia, cerebellum, thalamus and pons with progressive neurological defects

Charcot-Bouchard aneurysm

Vs. saccular berry aneurysm: associated with ADPKD, ehlers-Danilo’s syndrome and HTN in the circle of Willis from a subarachnoid hemorrhage. Sudden severe headache occurs with rare neuro defects

296

Treatment of oral candidiasis?

Nystatin= similar mechanism to Amphotericin B (binds ergosterol in cell membrane and causes pores and leakage of fungal contents), but is topical in form. Used for simple, localized candida infections

297

Strep pneumoniae is able to obtain new genetic material from the environment that is released following death and lysis of neighboring bacterial cells by a process called _______, via _________

Transformation which allows the bacteria to take up exogenous dna fragments directly and integrate them into its genome

This allows strep pneumonia strains that do not form a capsule to be taken up by those that do to become virulent (capsule=virulence for antiphagocytic activity)

298

when is potassium permeance highest in a neuronal AP?

Repolarization phase

299

As you age on PFTs, the total lung capacity _____, the forced vital capacity ______ and the residual volume ______

Stays the same (decreased chest wall compliance balances the increased lung compliance)
Decreases
Increases

Steady decreases in chest wall compliance from stiffening from rib calcification. Lung compliance increases with age, due to loss of elastic recoil in alveolar ducts

300

Child with no vaccination history presents with high fever, cough, coryza, conjunctivitis and small white spots with an erythematous base on the buccal mucosa. What is he most likely to develop of the next couple of days?
What is a complications days, and then years from now?

Maculopapular rash- measles. Starts on face and descends down like rubella, sparing the palms and soles. (Spots were koplik spots)

Pneumonia/secondary bacterial infections, encephalitis
Years- subacute sclerosing panencephalitis

301

Patient recently swimming in waters of Southeast Asia presents with intermittent GI and pulmonary symptoms and pruritic, erythematous, linear streaks on the thigh. How would diagnosis be made in a stool culture?

Finding rhabditiform larvae in the stool- hatched from the eggs of adult parasites seen on intestinal biopsy

Strongyloides stercoralis

302

what cytokine plays an important role in anti-inflammatory and immunomodulation, especially in IBD?
It attenuated the immune response by inhibiting TH1 cytokines, reducing MHC class II expression, and suppressed macrophages and dendritic cells

IL-10

303

Two distinct groups with varying levels of the same dose of isoniazid after metabolism for four hours is due to differing speeds of ______

Acetylation

Slow acetylators will also metabolize dapsone, hydralazine, and procainamide slowly.

304

Rupture of the chordae tendinae resulting in mitral regurg would give a _______ preload, _____ afterload, and resulting ______ ejection fraction.
How is this different from a chronic, compensated mitral regurg?

Increased preload: LV must accommodate the regurgitate blood volume in addition to the normal pulmonary venous return, increasing LVEDV
Decreased afterload: incompetent mitral valve forms a low resistance regurgitate pathway for blood flow into the LA, decreasing afterload.
These two together= increased ejection fraction

In chronic MR, compensatory LA dilation allows the LA to receive the regurgitate volume at lower filling pressures, preventing pulmonary edema. LV undergoes eccentric hypertrophy. Can still maintain ejection fraction with increased LVEDV, but there is no afterload increase until it becomes decompensated.

305

New born with Down syndrome evaluated shortly after birth due to a protrusion from umbilical cord site covered by skin, protrudes with increased abdominal pressure (crying), reducible, asymptomatic, and will resolve spontaneously
What is this defect due to?

Umbilical hernia- due to incomplete closure of umbilical ring

306

Severe arthritis in adult life, pigment deposits in connective tissue throughout the body (blue black sclera deposits, dark auricular cartilage, deposits in the spine causing ankylosis, etc) and urine turns black when exposed to air due to oxidation of _________

Homogentisic acid

This syndrome is called alkaptonuria- auto recessive cause by deficiency of homogentisic acid deoxygenase, which normally metabolized homogentisic acid into maleylacetoacetate.

307

what muscle is responsible for the upward traction on the medial fragment of the patient’s fractured clavicle? It will pull the clavicle superiorly and posteriorly.

sternocleidomastoid muscle

308

Enhancer sequences bind activator proteins that ________
Enhance sequences are located where?

Facilitate the bending of DNA, which allows activator proteins to interact with general transcription factors and RNA pol II at the promoter to increase the rate of transcription

Upstream or downstream of the gene being transcribed, within the introns being transcribed or on separate chromosomes

309

most common type of heart defect in Down syndrome?
In DiGeroge syndrome?

Complete AV canal defect- failure of endocardial cushion fusion

Tetralogy of Fallot, truncus arteriosus, transposition of the great arteries

310

12th rib laceration can puncture ______

Kidney*

Ribs 9, 10, and 11 overlie the spleen

311

Patient with symmetrical peripheral neuropathy of the distal extremities, with resulting sensory and motor impairments, cardiomyopathy, peripheral edema, tachycardia is categorized by what vitamin deficiency?

Thiamine deficiency- wet beriberi

312

Rash with Target lesions with a dusky central area, a dark red inflammatory zone surrounded by a pale ring, and an erythematous halo. Most commonly on extremities, trunk and neck, but severe cases can involve oral mucosa and tongue

This rash is typically associated with?

Erythema multiforme

Typically associated with infections- like HSV and mycoplasma

313

Amniotic fluid that enters maternal circulation via sites of uterine trauma or cervical lacerations results in ______ due to AF containing ______ metabolites and release of ____
What would this cause?
Histology/biopsy of maternal lungs?

Anaphylactic reaction due to arachidonic acid metabolites in the AF and release of tissue factor

Occlusion and vasospasm of maternal pulmonary circulation, leading to LV failure, decreased CO, and VQ mismatch leading to cardiopulmonary arrest
Release of tissue factor causes DIC

Amniotic fluid embolism would cause biopsy to show fetal squamous cells and mucin in the maternal pulmonary arteries

314

Vitamin deficiency/overuse (pick one) of ________ causes hepatosplenomegaly, hyperlipidemia, dry skin, alopecia, visual difficulties/papilledema

Vitamin A overuse

315

Protection from influenza infection by either vaccination or prior seasonal exposure of a similar strain is mediated most importantly by antibodies to _____

Hemagglutinin humoral response is typically the most important source of protection, blocking its binding to the host cell

316

Thiazolidinediones exert their glucose lowering effect by ______
They bind to the ______, which is what type of receptor?
What gene is regulated by this receptor?

Improving insulin sensitivity

PPAR-gamma, which is an intracellular nuclear receptor
Regulates adiponectin, a cytokine secreted by fat tissue that enhances insulin sensitivity and FA ox.

317

Priapism is a serious side effect of what antidepressant?

Trazadone- highly sedating typically used for insomnia

318

_____ proliferation is associated with mutations in KIT receptor tyrosine kinase. This proliferation occurs in the bone marrow, skin and other organs and cause excessive _____ release

Mast cell; histamine

Symptoms of systemic mastocytosis- syncope, flushing, hypotension, pruritus, urticaria, gastric acid secretion (which can then inactivate pancreatic and intestinal enzymes causing diarrhea)

319

What opioid is contraindicated in patients who have been on long term opioid therapy, because it can precipitate withdrawal symptoms?
MOA?

Buprenorphine- partial opioid receptor agonist that binds with high affinity but has low intrinsic activity

320

Esophageal motility disorder characterized by reduced numbers of inhibitory ganglia cells in the esophageal wall, favoring excitatory ganglia is called ______
____ contraction of UES, ______ amplitude of peristalsis in the mid esophagus, and _____ tone and incomplete ______ at the LES

Achalasia

Normal
Decreased
Increased tone and incomplete relaxation

321

What bugs are associated with a septic abortion- retained products of conception that become infected
Symptoms: fever, abdominal pain, uterine tenderness and foul smelling vaginal discharge

What is a long term complication of this?

Staph aureus
Gram negative bacilli- E.coli and group B strep.

Asherman syndrome- adhesions in the uterine cavity that can lead to secondary amenorrhea and infertility

322

Patient started on infliximab and 10 days later develops joint pain and a pruritic skin rash. Skin biopsy shows fibrinoid necrosis and neutrophil infiltration of small BVs. What immunological marker corresponds with this condition? Decreased/increased serum _______ levels

Decreased serum C3 levels

Suggestive of serum sickness- type III HS
Deposition of IgG or IgM complement fixing antibodies results in localized complement consumption and hypocomplementemia

323

Treatment for malaria includes chloroquine and primaquine for different reasons. What is each’s purpose?

Chloroquine eradicates C-sensitive plasmodia from blood, but has no activity on latent hepatic infections by P vivax and P ovale
Primaquine must be added to the regimen to completely eradicate the hypnozoites and prevent latent infection/relapse

324

Patient presents with dyspnea, cough, swelling of the face, neck and upper extremities, headache (cerebral edema/ICP), dilated collateral veins in upper torso
Patient will most likely have what causing this syndrome?

Superior vena cava syndrome

Usually from mediastinal mass- lung cancer or NH-lymphoma

325

What AB is most specific for diagnosing RA?

Anti-cyclic citrullinated peptide (anti-CCP)

RF has poor specificity- found in healthy patients, SLE, and those with mixed cryglobulinemia

326

The prostatic plexus lies within the fascia of the prostate and originates from the ______. The lesser and greater _______ nerves arise from the plexus and pass beneath the pubic arch to innervate ______ and _______

Inferior hypogastric plexus
Lesser and greater cavernous nerves innervate the corpora cavernosa of penis- damage can cause erectile dysfunction

327

elderly patient with fever, morbilliform rash on trunk and arms, and encephalitis associated with flaccid paralysis. Lumbar puncture shows viral RNA

West Nile virus- flaviviridae, ss positive sense RNA transmitted by mosquitos in summer months

328

Adverse reaction to antipsychotic medication characterized by severe lead pipe rigidity of muscles, hyperthermia, autonomic instability and mental status changes

NMS

Different from SS because NMS does not exhibit hyperreflexia and clonus
Acute dystonia will not produce mental status changes and hyperthermia, and typically only distressing contractions in the neck

329

intraventricular hemorrhage is common in what patients and usually originates from ______, a highly vascularized layer in the subventricular zone from which neurons and glial cells migrate out during brain development

Premature infants
Germinal matrix

330

Coronary dominance is determined by the coronary artery that supplies blood to the ______ artery and usually supples what?

Posterior descending artery (posterior IV artery)
70% will be RCA
10% will be left circumflex artery or can be codominant

The AV nodal artery usually arises from the dominant coronary a

331

Patient with ph>4.5, clue cells, and positive amine odor with KOH whiff test can be treated with what TWO meds

Metronidazole OR clindamycin

332

most common urea cycle disorder resulting in excess carbamoyl phosphate, which stimulates pyrimidine synthesis. Orotic acid accumulates and shows up in the urine. Patients will have hyperammonemia, which can cause episodes of vomiting and confusion, tachypnea occurs from cerebral edema from ammonia build up, resulting in central hyperventilation and respiratory alkalosis

Ornithine transcarbamylase deficiency

333

Cells found that are suggestive of prior episodes of pulmonary congestion and edema that arose due to heart failure, caused by erythrocytes extravasating into the alveoli due to increased intravascular pressures

Hemosiderin containing macrophages of alveoli (heart failure cells)

334

Patient with recurrent bacterial and fungal infections: Dihydrorhodamine flow cytometry assess the production of of _______ by measuring the DHR to rhodamine, a fluorescent green compound detected by flow cytometry. Cells with deficient ______ activity will have decreased fluorescence

What is another test to assess this disease?

Superoxide radical production
NADPH oxidase activity

Chronic granulomatous disease can also be assessed by nitroblue tetrazolium tests. If radical production is normal, it will reduce the yellow NBT to dark blue within the cells.

335

What type of bias is an apparent increase in survival time among patients undergoing screening when they actually have an unchanged prognosis?

Lead time bias

336

Substance X is freely filtered by the glomerulus, but majority is secreted by the PCT and is not reabsorbed by any portion of the nephron

PAH

337

Administration of morphine followed by acute pruritus is likely from what type of mast cell activation

IgE-independent mast cell degranulation

Activate PKA and PI3 kinase to induce mast cell degranulation- also opioids, radiocontrast, and vancomycin.

338

T(14;18) results in overexpression of ______ in what lymphoma
T(8;14) results in overexpression of _____ in what lymphoma

Bcl-2 in follicular lymphoma
C-myc in burkitt’s lymphoma

339

Orthomyxoviruses, like influenza viruses, contain what type of genome to allow them to perform ________

Vs. antigenic drift?

Segmented genome for genetic reassortment (when 2 distinct strains infect the same cell)

Point mutations on HA and NA to slightly alter product proteins and increase infectivity/evade immune system

340

Glycosyltransferase enzyme is a crucial component of _______ in bacteria
AB directed against this enzyme would not work on _______

Peptidoglycan synthesis- adds glycan molecules to the growing polypeptide chain

Mycoplasma because it completely lacks a cell wall. So it is treated primarily by inhibition of bacterial ribosomal function (macrolides)

341

Previously healthy male with new onset Progressive heart failure (decompensated due to the presence of bibisailar crackles and JVD and peripheral edema) in the setting of a recent viral infection should indicate ________

Dilated cardiomyopathy due to viral myocarditis from direct viral injury
Eccentric hypertrophy with decreased ventricular contractility (systolic dysfunction)

342

Middle aged female presents with menorrhagia and dysmenorrhea, a uniformly enlarged uterus*, negative hCG, normal appearing endometrial tissue on biopsy, and a history of a bilateral tubal ligation procedure.

Adenomyosis- presence of endometrial glandular tissue within the myometrium

343

Triad of congenital hydrocephalus, intracranial calcification, and chorioretinitis

Congenital in utero infection of Toxoplasmosis

344

The superior thyroid artery comes from ______ and courses with what nerve
What is the only muscle innervated by this nerve?

From the external carotid artery
Courses with the external branch of the superior laryngeal nerve

Cricothyroid - tenses the vocal cords
Injury can cause a low, hoarse voice with limited range of pitch

345

Patient with paroxysmal supraventricular tachycardia. Rapid IV admin of _____ results in instantaneous resolution and is accompanied by flushing, burning in the chest (due to bronchospasm), hypotension and shortness of breath

Adenosine- coronary arteriole vasodilator and affects cardiac conduction by hyperpolarizing the nodal pacemaker to briefly block conduction through the AV node
Rapid onset, short lived

346

What TB medication can cause a sideroblastic anemia?
MOA of this?

Isoniazid- inhibits pyridoxine phosphokinase, which normall converts B6 to its active form, which is a cofactors for ALA synthase in the RL step of heme synthesis

347

Calculating t1/2=

(0.7xVd)/clearance rate

348

Patient with cafe au lait spots and multiple raised fleshy tumors: these Cutaneous neurofibromas are composed of _______ embryologically derived from _______

NF-1

These benign nerve sheath neoplasms are Composed of Schwann cells. Derived from neural crest cells

349

central lines are typically placed where?
*hint: derived from the common cardinal veins

IJV or subclavian vein and advanced until the tip enters to SVC, which is derived from the common cardinal veins

350

Differential clubbing and cyanosis in lower extremities without BP or pulse discrepancy are pathognomonic for _____ and complicated by_______, pulmonary HTN causing a reversal of shunt flow from left-to-right to cyanotic right-to-left

Patent ductus arteriosus (distal to the L subclavian artery)
Eisenmenger syndrome

351

Vasopressin increased the absorption of a specific solute that is important for generating a high medullary concentration gradient. What is the solute and where does vasopressin act to do this

Medullary collecting duct
Increases passive urea transporters here to leak out into the medullary interstitium and promote further water absorbing capacity of the nephron

352

SAH sequelae causing altered mental status or neuro focal signs is caused by?
Can be prevented by?

Vasospasm- altered mental status and focal neuro signs
Calcium channel blockers can be used as a preventative agent after a SAH

353

what is the MOA of Amphotericin B’s AE?

It normally binds ergosterol cell membrane of fungi, but has some degree of binding to cholesterol in the human cell
Nephrotoxicity, anemia, thrombophlebitis

354

Meningitis bug that is resistant to cefotaxime (third gen cephalosporins)? MoR?
What type of immunity is required to clear this infection?

Listeria- due to altered PBPs but it is susceptible to ampicillin

Cell mediated immunity- watch those who are deficient

355

Opportunistic yeast stained on India ink with a thick capsule causing meningitis should be treated with what initially, then what long term?

Amphotericin B initially
Then long term fluconazole

356

What enzyme is deficient in a patient with fair skin, blue eyes, musty body odor, and tremors/seizures, and intellectual disability?
What AA is essential?

Deficiency of phenylalanine hydroxylase- which converts Phe to tyrosine, which becomes essential in these patients

357

Patient returns from a trip to Brazil that caused acute fever, headache, retro-orbital pain, and joint/muscle pain, and a diffuse maculopapular rash. Also has signs of hemorrhage and thrombocytopenia

What has the same mode of transmission?

Dengue fever- Aedes aegyptimosquito

This mosquito also transmits the virus causing chikungunya fever

358

Path of CSF absorption after the fourth ventricle
What pathology occurs if this final absorption step is disrupted?

Into the formation of Luschka and Magendie—>SAS. In the SAS, CSF is absorbed by arachnoid granulations and then enters the venous sinuses.
If this path to the SAS is disrupted, then non-communication hydrocephalus occurs. But if there is no obstruction and damage to the SAS villi is done by infection or SAH, it results in communicating hydrocephalus and all ventricles are symmetrically enlarged

359

Patient with high risk of CVD and normal lipid levels and slightly low HDL should be placed on what medication?

Statin- lower LDL cholesterol and raise HDL cholesterol. Most protective in preventing CV events, regardless of baseline lipid levels

*use of meds to solely raise HDL cholesterol do not improve CV outcomes (like niacin)

360

differentiating between bipolar I with psychotic features and psychotic disorders

Depends on severity of mood symptoms and temporal relationship between the mood disturbance and psychosis
Is psychotic disorders, mood symptoms are not a prominent feature
In bipolar disorder, the severe mania and delusions occur together exclusively

361

3 nerves that innervate the external ear
Nerves of the external auditory canal

Great auricular nerve- C2-C3
Auriculotemporal nerve of V3
Lesser occipital nerve- C2-C3

V3 auriculotemporal nerve mostly
*the posterior part of the external auditory canal is innv by the auricular branch of the vagus nerve (stimulation here can trigger a vasovagal response if irritated)

362

What enzyme is responsible for removing short fragments of RNA primers that are base paired to the DNA template, utilizes 5’-3’ exonuclease activity and replaces the primers with DNA

DNA pol I- only one that has a 5’-3’ exonuclease activity

*DNA pol III has 3’-5’ exonuclease activity for proofreading while it elongates the DNA chain via a 5’-3’ polymerase but cannot remove primers

363

Flow volume curve in a lung that has destruction of interalveolar walls and/or narrowing of the bronchi

COPD- the decreased lung elasticity results from the destruction of interalveolar walls (emphysema) or narrowing of bronchi (chronic bronchitis)

364

What is the mechanism by which eosinophils defend against parasites?
Vs. eosinophils in Type 1 HS reactions?

Antibody dependent cell mediated cytotoxicity:
Eosinophils proliferation and activation is stimulated by IL-5 produced by TH2 and mast cells. The parasite is coated in IgE and G ABs and bind the Fc receptors on the eosinophils, which triggers degranulation and release of MBP and ROS which kill the parasite

Eosinophils synthesize PGs, LKTs, and cytokines that produce inflammation seen in late phase type 1 HS and chronic allergy reactions

365

What shares its embryological origin with the thymus?
What pouch does this come from?

3rd pharyngeal pouch- thymus and inferior parathyroid glands

366

Oxybutynin vs. Bethanechol action on bladder

Oxybutynin is used for urinary incontinence- antimuscarinic agent
Bethanechol is used for post surgery urinary retention- muscarinic agonist

367

Patient with perifollicular hemorrhages, myalgia, subperiosteal hematoma, petechial hemorrhages, and gingivitis-
Due to a deficiency in:

Scurvy
Ascorbic acid (vitamin C)

Collagen defects in BV walls lead to widespread micro vascular bleeding

368

Patients presents after a camping trip to New Mexico with fever, agitation, and disorientation, seeing/hearing things that do not exist, drools excessively, painful throat spasms where he is avoiding water, mydriasis and neck rigidity
Which acts as an entry receptor for the causative agent?

Muscle nicotinic Ach receptors- rabies
Most likely came into contact with contaminated saliva on the camping trip
Travels in a retrograde fashion through peripheral nerve axons to the DRG then rapidly ascends into hippocampus and brain stem

369

What structure limits ascent of a horseshoe kidney?

IMA

370

UTI- G- lactose fermenter, indole-positive rod.

E.coli

Indole + differentiates it from Enterobacter (ability to convert tryptophan to indole)

371

High intraplaque activity of ______ most likely results in a ruptured plaque (ACS)?

Stability largely depends on the mechanical strength of the fibrous cap- thinner=more unstable
The balance of collagen synthesis and degradation determines the mechanical strength of the cap. Activated macrophages infiltrate and contribute to breakdown of collagen by secreting: metalloproteinases

372

during surgery for an appendicitis, what can be used as a surgical landmark?

Teniae coli
Continuous layer of longitudinal muscle that surrounds the rectum just below the serosa. This layer condenses to form 3 distinct longitudinal bands that will eventually converge at the root of the vermiform appendix

373

Isolated systolic HTN in a patient >60 most likely from:

Increased aortic stiffening
Can also result from an increase in CO due to severe aortic regurg

SBP >140 with a normal diastolic BP

374

Sudden onset palpitations and rapid regular tachycardia is consistent with ____ which is most often due to a
How does a carotid sinus massage alleviate symptoms?

Paroxysmal supraventricular tachycardia
Due to a reentrant impulse traveling circularly between the slowly and rapidly conducting AV node segments

The carotid sinus reflex has afferent limbs arising from baroreceptors that travel up with CN X and IX, whereas the efferent limb carries parasympathetic impulses to the SA and AV node via CN X.
Massage leads to increased firing which increases vagal parasympathetic tone to slow the conduction through the AV node and prolong its refractory period.

375

HTN, hematuria and moderate proteinuria with a renal biopsy that shows linear deposits of immunoglobulin along the glomerular BM. Can cross react with the pulmonary alveolar membrane
What are these deposits made up of? What are these antibodies targeting? What would light microscopy look like?

IgG and C3. The anti-GBM ABs target collagen type IV, leading to subsequent complement deposition.
This results in a form of rapidly progressive (crescentic) GN
Glomerular crescents composed of proliferating parietal cells with an infiltration of monocytes and macrophages are seen on LM

Goodpasture’s syndrome

376

Lifecycle of hepatitis B virus- the genome is a ______ molecule that replicates through ______
The genome serves as a template for transcription into a _______
This strand in turn serves as a template for _______ and _______

It is a partially double stranded DNA molecules that replicates through reverse transcription
After virion entry and nuclear import, the ds circular DNA serves as a template for transcription into an intermediate +ssRNA
This RNA acts as a template for translation of viral proteins and reverse transcription into a ss DNA intermediate, which is converted back into a partially ds DNA.

Overall: dsDNA—>+RNA template—>ds DNA progeny

377

Man in a recent MVA with subsequent hyperosmotic volume contraction, where the loss of free water exceeds the loss of electrolytes, resulting in increased osmolarity and contracted volumes in BOTH the ICF and ECF

Vs. the effect of an acute hemorrhage/diarrhea

Central DI

Hemorrhage/diarrhea would cause an isotonic loss of ECF volume, with no effects on osmolarity or ICF

378

MOA of celecoxib?

Selective COX2 inhibitor- potent anti-inflammatory effects with less risk of bleeding and GI ulcers

379

A neonate presenting with macrocephaly, bulging fontanelle, poor feeding, DD and ______

Muscle Spasticity and hyperreflexia- due to upper motor neuron injury caused by stretching of periventricular pyramidal tracts

380

Antifungals that inhibit the demethylation of lanosterol into ergosterol in fungal cells. Suppress synthesis of ergosterol. Also inhibit cytP450

Azoles*

381

A study where the unit of analysis is comparing populations instead of individuals is called

An ecological study

382

Antibiotic of choice for treating lung abscesses (typically in alcoholics aspirating their own normal flora- anaerobic and aerobic)

Clindamycin

383

Calculation of the net filtration and excretion rate:
Inulin is-

Total filtration rate=inulin clearance x plasma conc of substance A
Net excretion rate=inulin clearancexplasma conc - tubular reabs of substance A

Inulin is neither secreted nor reabsorbed so the GFR is approximately equal to inulin clearance

384

Anti-Jo1 antibodies are specific for:

Dermatomyositis

385

Anaphylaxis results in widespread mast cell and basophilic degranulation, resulting in release of ____ and ____

What mechanism triggers vasoactive substance release by these cells?

Histamine and tryptase (specific to mast cells)

High affinity IgE receptor on mast cells binds the Fc portion of IgE. When a multitalented antigen comes in contact with the mast cell, multiple IgE antibodies become cross linked, resulting in aggregation of the receptors. This activates TK signaling and degranulation

386

Trisomy that results in a defect of fusion of the prechordal mesoderm, resulting in midface, eyes, forebrain abnormalities and other midline defects like holoprosencephaly, microcephalic, cleft lip/palate, and omphalocele

Patau syndrome- trisomy 13

387

Ectopic pregnancy uterine curretage appearance/findings of the tissue?

Uterine specimen would reveal decidualized endometrium only, consistent with dilated, coiled endometrial glands and vascularized edematous stroma
These changes occur in a luteal phase, under the influence of progesterone as the endometrium prepares for implantation

388

What is the location of a femoral nerve block?

Inguinal crease at the lateral border of the femoral artery
L2-L4 from lumbar plexus and emerges between psoas and iliacus muscles passing under the inguinal ligament lateral to the femoral artery. Not in the femoral sheath.

389

A t(14;18) chromosomal translocation causes what genetic abnormality/overexpression? What disease?

Bcl2 overexpression- follicular lymphoma
B cell lymphoma 2 protooncogene (anti-apoptotic) is moved near the immunoglobulin heavy chain enhance element on 14.

390

Mammography shows microcalcifications and features of breast tissue that are distended by pleiomorphic cells with prominent central necrosis without extension beyond BM

DCIS

391

Infusion of isotonic saline causing an increased BP and LV volume engorgement will cause a release of what peptide hormones?

Intravascular volume expansion with an increase in intracranial volume filling pressures will lead to stretching of myocardium and release of ANP and BNP- for naturesis (increased GFR) by activating GC—> cGMP promoting glomerular arteriolar vasodilation and efferent constriction.
Also inhibit sodium reabs and renin secretion

392

Metyrapone stimulation tests for:
Mechanism

Adrenal insufficiency
Inhibits cortisol synthesis (inhibits 11 beta-H), low cortisol stimulates the HPA axis to create a surge of ACTH, and normally the adrenal glands will put out 17-h corticosteroids that will accumulate in the urine

393

What drugs typically cause increase in systolic and diastolic BP and a reflexive, delayed decrease in HR?

Selective alpha 1 agonists- phenylephrin and methoxamine
Vasoconstriction and increased BP, stimulation of baroreceptors in carotid sinus and aortic arch causes a reflexive increase in vagal tone on heart which results in inhibition of pacemaker activity of SA node, slowed conduction to AV node, and decreased contractility

394

Young women with menstrual cycle irregularities, heavy bleeding, long periods, without pain is due to:

Immature hypothalamus pituitary ovarian axis- causing anovulatory cycles- causing estrogen to remain high, no progesterone surge, causing the endometrium to remain in the proliferative stage

395

After HIV enters a host cell, it releases viral RNA and first:
What do integrase inhibitors do?

Transcribes dsDNA by reverse transcriptase. Then enters the nucleus and inserts itself into the host’s cell via integrase to become a provirus—- cell machinery makes a mRNA from it for further translation.
Integrase inhibitors would stop the formation of mRNA via transcription

396

Increased direct bilirubin in the blood with self resolving episodes of jaundice. Biopsy shows abundant pigment inclusions in lysosomes of liver and otherwise normal hepatocytes. Pigment is composed of polymers of epinephrine metabolites

Dubin Johnson syndrome- defective excretion of bilirubin glucuronides due to mutation in the canalicular membrane transport protein. Liver appears grossly black due to impaired excretion of epinephrine metabolites that accumulate in lysosomes

397

Where in the LN is primarily populated by T cells?
What congenital disease would this location be poorly developed?

Paracortex- lies internal to the cortex, between the follicles and medulla. Dendritic cells present antigen from blood and lymph to T cells in this region
This region becomes enlarged during adaptive cell immune responses- viral infections.

DiGeroge syndrome- failure of 3rd and 4th pharyngeal arches

398

Two types of COPD
PFT testing will show what?

Bronchitis and emphysema
Obstructed PFTs- decreased FEV1/FVC due to exploratory airflow obstruction. Decreased FVC and increase in both TLC and RV due to destruction of inter alveolar walls, decrease in lung elastic recoil, and distal airspace enlargement


(Restrictive lung diseases will have reduced lung volumes and normal or increased FEV1/FVC ratio)

399

A gram negative coccobacillus that causes meningitis in neonates that must be plated with _____ on blood agar to grow. What factors does it require from this ^ to grow?

With staph aureus- supplies X factor (hematin) and V factor (NAD+) to support H. Influenza type b growth

400

Lymphogranuloma venerum is caused by what pathogen?

Chlamydia trachomatis serotypes L1-L3
Small gram negative intracellular bacterium- inclusion bodies
Characterized by a single painless ulcer that goes away then weeks later followed by swollen, painful, coalescing inguinal nodes (buboes)

401

How is 6-mercaptopurine activated? inactivated?

A cytotoxic purine analog that inhibits de novo purine synthesis
Activated by hypoxanthine-guanine phosphoribosyl transferase
Inactivated by xanthine oxidase and thiopurine methyltransferase in liver

*watch doses is also on allopurinol, an XO inhibitor

402

What characteristic of thrombi in a coronary artery allow for compensation by the development of arterial collaterals around the point of occlusion, resulting in less damage than expected to the occluded vessel?

Slow growth of the clot

403

Enoxaparin is a form of what medication?

LMW heparin- binds antithrombin III to take up thrombin and Xa and anticoagulate

404

The goal of _____ therapy is to dissociate the bedroom from any stimulating activities that do not involve sleep (reading, TV, eating) and the fear of not sleeping in patients with insomnia

Stimulus control therapy- example: leave the bedroom if not able to fall asleep within 20 minutes

405

Glycogen is the primary source of glucose for the first ____ hours of fasting, then GNG takes over.
During GNG, the first committed step is:
What step allows bypassing of pyruvate synthesis to get to glucose? (Because of its unidirectional enzyme)

12-18 hours

Biotin dependent carboxylation of pyruvate to oxaloacetate by mitochondrial pyruvate carboxylase. Oxaloacetate must bypass pyruvate kinase by being converted to malate, shuttled to cytosol, then converted back. Then OAA—>phosphpoenolpyruvate to go to glucose

406

Eukaryotic flagella and cilia are composed of a central core known as the axoneme, which is made up of _____ doublets in a ____ orientation. Each doublet is connected to the adjacent one by _____ and when these are mutated is forms what pathology?
The axoneme is anchored to the cell by a:

Microtubules in a 9+2 arrangement
Connected by dyenin arms- causes Kartagener syndrome that impairs ciliary function
Basal body connects axoneme to cell

407

High dose steroids can cause corticosteroid-induced psychosis and what affect on leukocytes and vascular endothelial cell?
On stomach?

Neutrophil count increase due to demargination of neutrophils previously attached to the vessel wall. Causes decreased neutrophil recruitment to fight infection in tissues

Peptic ulcer risk or GI bleed due to decreased PGEs

408

Radioactive iodine can cause what after nuclear explosions?
What is given prophylactically to combat the 131 radioactive iodine?

Radiation induced hypothyroidism and thyroid carcinoma.
Potassium iodide- protects thyroid from accumulation of radioactive version. The Na/iodide symporter on basolateral membrane of follicular cells takes up all forms of iodide, so high serum levels of non radioactive iodide can competitively inhibit the radioactive version.

*too large of iodide levels can actually inhibit iodide organification and reduce TH synthesis

409

Only pathogenic fungus that has a polysaccharide capsule, which appears red on mucicarimine stain and as a clear unstained zone with India ink. Methenamine silver stain can ID yeast form.
Typically causes pneumonia or meningencephalitis in immunocompromised or immunosuppressed hosts

Cryptococcus neoformans

410

Meningitis that shows CSF fluid with hemorrhagic lymphocytic pleiocytosis with increased protein and normal glucose
On neuroimaging, classic finding includes the presence of abnormal
MRI signal in bilateral temporal lobes.
Treatment?

Acyclovir- HSV encephalitis
Nucleoside analog that competes for viral DNA polymerase to terminate the DNA chain

411

High arterial blood pH, high HCO3-, and high pCO2=
Reasons for this disease state and how to differentiate them via labs?

Metabolic alkalosis (Resp alkalosis would have low pCO2)
Vomiting or diuretic use would cause volume and Cl- depletion (saline responsive alkalosis) (low in urine and high in urine, resp.)
Also hyperaldosteronism can cause this but is saline unresponsive

*measuring volume status and urinary chloride can narrow down the cause

412

Increased LV volume and shifting of pressure volume curve to the right would be characterized by what?

Dilated cardiomyopathy- LV systolic dysfunction. (Increased compliance due to thinning of cardiac wall)
Alcohol, doxorubicin, selenium def, and viral myocarditis can all cause this

413

Conditions that increases LV EDP at the same/normal LVED volumes imply what?
Shift in pressure volume curve that goes upward and left
What is an important cause of this phenomenon?

Decreased compliance and diastolic dysfunction. HF with preserved EF.
HTN, obesity, and Restrictive cardiomyopathy- infiltrative disorders (example: transthyretin, a protein from liver that can accumulate and misfold with gene mutations, produces amyloid that infiltrates the myocardium)

414

TH1 vs TH2:
Type of cell immunity-
Function-
Substances secreted-
Result

Deficiency in TH1 activation would result in?

Cell mediated: humoral AB mediated
Activate macrophages and cytotoxic T cells: Act B cells and promote class switching
IL12, IFNgamma, lymphotoxin B: IL4, IL5, IL10 and 13
Cytotoxicity and delayed HS: secretion of ABs

*when macrophages present antigen to naive T cells, it produces
IL-12 and differentiates the T cell into TH1 subset.
If IL-4 is released by another antigen presenting cells, it differentiates into a TH2.

Without it, macrophages could not be activated by IFN gamma, and macrophages are necessary for delayed HS reactions and cytotoxicity against intracellular organisms, especially mycobacterium.

415

Which method is most successful in a Turner syndrome patient who wants to get pregnant?

IVF with a relative or donor ovum

416

Neural tube defects occur due to failure of fusion of the neural plate edges during 4th week of development. Fusion goes cranial to caudal. If either end does not fuse, this causes a leakage of what two markers that will be elevated in amniocentesis to indicate a potential failure of fusion of neural plate edges?

AchE and AFP
Used for detection of anencephaly and spina bifida

417

In a patient with severe cerebral edema, facilitating a drop in paCO2 with a ventilator would be sensed by the brain and cause what response?

Vasoconstriction—> reduced cerebral blood volume leads to decreased ICP.

Normally CO2 is a potent vasodilator in the cerebral vasculature.

418

Patient with a history of SLE and new complaints of high BP, progressive truncal obesity, facial plethora, hyperglycemia, and proximal muscle weakness?

Thinking Cushing syndrome from exogenous glucocorticoid use for autoimmune suppression
Would cause bilateral cortical atrophy from prolonged ACTH suppression

419

primary site of ribosomal RNA transcription in the cell?

Nucleolus.

420

A depolarizing NMJ blocking agent at the nAch receptor with AE of malignant hyperthermia in genetically susceptible adults and in severe hyperkalemia in patients with burns, myopathies, crush injuries and denervation.

Succinylcholine
In patients with burns, myopathies, crush injuries and denervation, the nicotinic Ach receptor is unregulated, so when succinylcholine is administered, it can cause a release of large amounts of potassium. Use vecuronium or rocuronium (non-depolarizing agents) in these patients.

421

Endocarditis from Staph Aureus in an IV drug user will most typically affect which valve? Heard best where?

Tricuspid regurg- think venous blood hitting right heart first.
Early systolic murmur heard best at fourth less intercostal space at left lower sternal border, Most accentuated with inspiration.

422

Major side effect to amiodarone due to its 40% iodine by weight?

Hyperthyroidism and hypothyroidism
CHECK TSH with amIODarone (iodine)

*class III anti arrhythmic in life threatening rhythm disturbance situations

423

Adjustable gastric banding placed around the cardia of the stomach would need to encircle what ligament?

Lesser omentum
Slows the passage of food to increase satiety

424

Lac operon regulation

Negatively by binding of repressor protein to operator locus
Positively by cAMP-CAP binding upstream from promoter region

Glucose present- decreases AC and then cAMP. So when glucose is around, lac operon is suppressed.

425

AA binding site on tRNA?
T loop on tRNA? D loop? (Arms of T)
Anticodon loop?

The acceptor stem of tRNA is created through the base pairing of the 5’terminal nucleotide with 3’. It contains the CCA tail, which is added to the 3’ end as a post transcriptional modification and serves as the AA binding site loaded on by aminoacyl tRNA synthetase

Binds ribosomes
Facilitates correct tRNA recognition by the synthetase

Contains sequences that are complementary to mRNA codon. During translation, it binds mRNA and assures placement of the proper AA in polypeptide chain.

426

Automaticity of pacemaker cardiac cells is controlled by what ion influx?

Calcium
Slow inward sodium current occurs during phase 4 of the AP, which brings the membrane potential closer to threshold, at which point phase 0 depolarization occurs due to a large increase in calcium influx.
Then phase 3 repol by outward potassium flux. Resting potential is phase 4.

Whereas a non pacemaker cell has phases 0-4. Phase 1 is the sharp depol by sodium influx, short repol by potassium, plateau phase by inward calcium, then full repol phase 3 to resting membrane potential phase 4 by potassium outflow.

427

UC is commonly linked with what chronic cholestatic disease?
Positive marker?

Primary sclerosing cholangitis- fatigue, pruritus, and scleral icterus with alternating strictures and dilations in bile ducts (onion skin fibrosis)
P-ANCA positive

**different from c-ANCA (granulomatosis with polyangitis)

428

Child with high fever but no clear source of infection must check for occult bacteremia. What cell clears a bacteremia infection?

Macrophages of the liver- Kupffer cells.

429

Slow rhythmic electrical changes noted in GI smooth muscle (slow waves) are generated by a unique mechanism involving?

Interstitial cells of cajal- producing rhythmic depol/repolarizations of muscularis propria independent of a stimulus
Basal electrical rhythm (pacemaker cell of the gut)
Stomach has lowest frequency with duodenum having highest freq

430

Familial syndrome with high risk of developing colorectal, GI tract, urinary and female reproductive tract cancers, with a few flat polyps in the cecum
Syndrome and genetic abnormality

HNPCC- auto dominant DNA mismatch repair gene mutations

431

Painless rectal bleeding in an elderly patient with a Hx of constipation or low fiber intake—> can progress to LLQ pain
After this episode has resolved, what diagnostic study should be done?

Diverticulosis—>diverticulitis
With marked leukocytosis and iron deficient anemia, want to rule out malignancy- perform a colonoscopy AFTER diverticulitis is controlled (so don’t cause a bowel perforation)

432

what artery can be compromised during an AAA repair?
What structures will be compromised if BV is damaged?

IMA- distal one third of transverse colon, descending, sigmoid and upper portion of rectum

433

Child with facial and neck swelling, persistence of an abdominal protrusion, and short limbs in a 3month old photo, parents immigrated from Indonesia
Child develops progressive intellectual disability and growth delay
Bone age is LESS than chronological age

Thyroid hormone deficiency- Thyroid hormone has a permissive affect on GH, specifically on bones, and maturation of CNS. So both would be underdeveloped

434

Genital lesions that are small papules that rapidly evolve into painful ulcers with erythematous base that bleeds with manipulation. With painful swollen LNs unilaterally

Chancroid- H. Ducreyi
Gram negative coccobacillus

435

Primary biliary cirrhosis treatment and positive lab

Ursodeoxycholic acid
Positive for anti-mitochondrial ABs

436

Portion of testes where sperm acquire motility has what kind of epithelium ?

Epididymis- pseudostratified columnar epithelium with stereocilia

437

Hepatitis C addition regimen with AE of severe depressive/neuropsychiatric complications, flu-like symptoms, -penias, alopecia, and interstitial fibrosis

Peginterferon alpha

438

Persistent headache, fever, mild cervical lymph node enlargement, and agglutination of horse erythrocytes when mixed with patient serum
Describe the pathogen

Explains positive mono spot test- EBV
A enveloped, ds DNA herpesvirus

439

Oxytocin is stored where

Herring bodies of posterior pituitary
(Made in paraventricular nucleus of hypothalamus)

440

A child presents much lower in height and weight than her age group, limbs are shorter than her torso, and head is relatively large.
Inheritance?

Autosomal dominant disorder- achondroplasia
Gain of function genetic defect in FGFR3- abnormality in cartilage formation and endochondral bone formation
Positive correlation between advanced paternal age (like other auto dominant disorders)

441

Child with altered mental status, nausea and vomiting, elevated anion gap metabolic acidosis
Can also have polyuria, polydipsia, abdominal pain, abnormal breathing- tachypnea followed by rapid deep breaths.
Management?

DKA - presenting symptoms of type I DM
Fluid resuscitation and insulin. *caution insulin shifts K into the cells and correction of acidosis will lead to a further intracellular shift in K. Must avoid life threatening hypokalemia*

442

Presents with fatigue, weight loss, depression, kidney stones -(hypercalcemia)
Decreased libido and peripheral vision- prolactinoma
Duodenal ulcers - gastrinoma, giving zollinger-Ellison syndrome
Mode of inheritance?

MEN type 1- auto dominant
Tumors of the 3 P’s- parathyroid, pituitary gland and pancreas

443

Gross specimen of GI tract- linear ulceration along bowel wall, wall thickening, creeping fat, and luminal narrowing/strictures, skip lesions, cobblestone mucosa and deep fissures
How would this present histologically?

Crohn’s disease- transmutation inflammation and noncaseating granulomas

Grossly- as compared to pseudopolyps in UC
Histologic UC- plasmacytosis, crypt abscesses, mucosal erosion, and mucin granulomas.

444

Pregnant woman presenting with nausea, vomiting, fever, abdominal pain and jaundice with recent travel history to India.
Genomic structure of the virus?

Fulminant hep E- single stranded, linear RNA virus
Transmitted via fecal oral route

445

Treatment for BPH vs. prostate cancer

BPH- alpha antagonists and alpha reductase inhibitors
Cancer- GnRH agonists

446

Dysphagia to both solids and liquids (indicating a diffuse cause rather than local obstruction), history of reflux and constipation, coupled with thickened skin and arthralgias
Common in African American women
What causes the esophageal symptoms?

Scleroderma- autoimmune CT disorder affecting GI, lungs, MSK and eventually the heart, vessels and kidneys.

Upper third esophagus is made up of striated muscle, middle has both, and lower third is smooth muscle. Develop dysphagia secondary to atrophy of smooth muscle in lower 2/3rd of esophagus and incompetence of LES

447

Major complications of pre-eclampsia seen on lab values

DIC- elevated D diners, PT, PTT, and platelet count
Hemolysis- increased LDH and bilirubin
Elevated liver enzymes

448

And MAOI and antibiotic used for treatment of gram positive bacteria that are resistant to other bacteria
Linked to serotonin syndrome- tachycardia, HTN, dilated pupils, hyperthermia, agitation, clonus, autonomic instability and hyperreflexia

Linezolid

449

Abnormally strong adherence of placenta to uterine wall because the placental villi attach directly to the myometrium due to defect in decidua basalis layer
Manifests as incomplete separation of placenta after delivery and can cause severe postpartum hemorrhage

Placenta accreta- most commonly involves a placenta previa after a prior C-section delivery

450

Antepartum hemorrhage after 20 weeks’ gestation
Occurs when the placenta overlies the internal cervical os
Absence of pain
Bright red blood

Placenta previa

451

Presentation of diabetic ketoacidosis, orbital pains, headache, and acute sinusitis
Fungal proliferation in walls of blood vessels and infarction/necrosis of tissues
Right angle branching and ribbon like nonseptate hyphae

Invasive mucormycosis - Mucor or Rhizopus species
Treat with amphotericin B

452

In cases of gastroenteritis due to infection by Salmonella enteritidis, symptoms are prolonged by what treatment?

Antibiotic treatment and can increase the rates of relapse
(S. Typhi infections SHOULD get antibiotics)

453

Charcot triad of RUQ pain, jaundice and fever
Leukocytes with neutrophilia and elevated band cell count

Bacterial cholangitis

454

Eczemtaous lesions that are painful and itchy on nipple and areola
Can be crusted, ulcerated, with serosanguineous discharge
Histology- presence of large cells with clear cytoplasm surrounding hyperchromatic nuclei “halo cell”

Paget’s disease of the nipple- usually an underlying ductal carcinoma

455

Skin lesions that are pruritic, erythematous, scaly and demonstrate a central clearing. Associated with athletic gear.
Systemic therapy? AE to medication?

Tinea corporis
Ketoconazole- inhibits CYP450scc and 17,20-desmolase/17alpha hydroxylase which causes anti-androgenic effects (decreased libido, impotence, and gynecomastia). Desmolase inhibition causes darkening of skin due to decreased cortisol levels causing increased POMC—> melanocyte stimulating hormone and ACTH

456

Premature separation of placenta from uterine wall usually during third trimester
Manifests as painful vaginal bleeding that is dark red (venous blood) and initiation of contractions can be seen.
Risk factors include smoking, hypertension and cocaine use during pregnancy

Abruptio placentae

457

where is ADH produced?

Supraoptic nucleus of hypothalamus

458

Deficiency of acetyl-CoA carboxylase with slow growth, unsteadiness, inability to use eating utensils, muscle hypotonia and myopathy.
What substance is likely deficient?

Dysfunction in fatty acid synthesis- rate limiting step is acetyl-CoA carboxylation and biotin to form Malonyl CoA, which is a substrate for FA synthesis
Malonyl CoA also inhibits carnitine shuttle from transporting FA into mitochondria for beta oxidation

459

Lipid lowering agent that activates the peroxisome proliferator-activated receptor-alpha (PARalpha) to increase activity of lipoprotein lipase for TG clearance
Usage is associated with gallstones, by reducing cholesterol solubility and bile acid synthesis

Gemfibrozil

460

Most common benign parotid tumor and contains multiple cell types, typically epithelial cells in a chondromyxoid stroma

Pleomorphic adenoma

461

patients with cholelithiasis are at risk fo developing BLANK due to gallstone ileus
Initially, bowel sounds can manifest as hyperactive and develop to hypoactive later.
Upright plain radiograph reveals air-fluid levels

Small bowel obstruction

462

Hepatic encephalopathy treatment mechanism

Poor liver function are unable to convert ammonia to urea
From a buildup of ammonia acts as a toxin to CNS
Lactulose is first line treatment- colonic bacteria convert it to lactic acid and acetic acid, which acidifies the colonic contents, which converts ammonia to nonabsorbable protonated form, ammonium, for excretion in the colon.

463

BRCA1 and 2 chromosome mutations
What disease shares a mutation on the same chromosome as BRCA1

17 and 13, resp.
NF1

464

What form of Chlamydia is the infectious form that allows it to attach to host cell and enter them?

Extracellular elementary body

465

AE from feedback loop with omeprazole usage

PPIs effectively raise the intragastric pH, leading to decrease in somatostatin levels and a downstream 2-4x rise in gastrin leading to atrophic gastritis
Other AE: b12 def, weakened bones, and increased risk of C.diff and pneumonia

466

Isolated direct hyperbilirubinemia with no other symptoms
Liver appears black grossly

Dubin Johnson syndrome- auto recessive disorder that results in defective excretion of conjugated bilirubin from liver

467

Vulvovaginal candidiasis is generally detected in a normal vaginal pH range of:
Whereas bacterial vaginosis is generally associated with:
Normal vaginal pH:

Less than 4.5
5.0-6.0
3.8-4.5 is normal maintained by Lactobacilli

468

History Constipation, crampy pain, and tenderness in LLQ, diarrhea, and a low fever and blood in stool in older patients
Described as inflammation of colonic diverticula, due to retention of material and fecalith formation most commonly in sigmoid
Risk factors are low fiber diet and obesity
Histologic presentatinon is:

Diverticulitis
A diverticula occurs when pressure causes herniation of colonic mucosa and submucosa through *attenuated (thinner) Muscularis propria, creating a false diverticulum.

469

Sore throat, fatigue, low grade fever, and malaise with non-puritic, red, discrete macules that progress to papular lesions that are non-tender and palms/soles and generalized LAD
If left untreated, it can progress to?

Secondary syphillis

Gummatous syphillis, cardiovascular (aortic arch or ascending aortic anuerysm—>infarction) and neurosyphillis

470

Presents with confusion, fever, nausea, hypotension, flank pain, and CT shows bilateral adrenal hemorrhage
Commonly caused by what?

Waterhouse-Friderischen: catastrophic adrenal insufficiency and vascular collapse due to hemorrhagic necrosis of adrenal gland
DIC is the most common cause due to menigococcemia from Neiserria meningitidis (gram negative diplococcal bacterium)

471

Young boy comes in with a straddle injury that causes urine to flow into the scrotum and perineal region. What portion of the urethra ruptured?

Rupture of the urethra below the urogenital diaphragm (at the junction between the membranous and penile urethra)

472

Cancers that spread hematogenously

HCC, renal cell carcinoma, choriocarcinoma, follicular thyroid carcinoma

473

HLA subtypes and their diseases:
HLADR3 and 4
HLADR3
HLA DR2
HLADR4

DM type 1
SLE
MS
RA

474

HLA subtypes for :
PAIR- psoriasis, ankylosing spondylitis, IBD, reiter syndrome

HLAb27 on chromosome 6p

475

Bacteria that can cause conjunctivitis 5-14 days after birth with yellow discharge and pneumonia
Intracellular inclusions
CBC eosinophilia

Chlamydia trachomatis

476

Treatment of UTI- reduced effectiveness if someone is taking mediation for history of GERD. Name each medication

UTI usually treated FQ, like levofloxacin
FQs are less effective with polyvalent cation usage- like in calcium carbonate for GERD

477

Urethritis, intracellular inclusions that are dark brown with few PMNs
Treatment

Chlamydia
Azithromycin or tetracycline

478

treatment for hypogonadism
SE

Androgenic steroids- premature closing of epiphyseal plates by promoting calcium deposition in bones

479

treatment of bacterial vaginosis with clue cells and foul smelling discharge

Gardenerella infection- metronidazole

480

Abdominal pain and constipation, diffuse and doesn’t change with meals, fatigue and difficulty concentrating, gingival lesions, iron def anemia
Can also have wrist and foot drop, hypertension, nausea, seizures, hearing loss
Treatment?

Lead toxicity
Dimercaprol or EDTA

481

Solitary thyroid nodule, dysphasia, hoarseness, increased serum calcitonin
With Recurrent kidney stones and hypertension
Histologically the thyroid nodule is polygonal to spindle sharped cells with granular cytoplasm in an amyloid filled stroma

MEN2- medullary carcinoma of thyroid

482

Pharyngitis, cervical lymphadenopathy, and fever.
Gram positive, rod shaped bacterium
This exotoxins production does what

Inhibits protein synthesis by ADP ribosylation of elongation factor 2.
C. Diphtheriae

483

Globozoospermia (sperm with round heads)- pathology and after correction what is the chromatid and ploidy of the cells?

Golgi apparatus is not transformed into the acrosome, resulting in male infertility because of incomplete spermiogenesis. Usually a series of post exotic changes marks final maturation.
Spermatic should are usually 23(1N,1C) 23 chromosomes and haploid

484

Prior to surgery for pheochromocytoma, what is the best medical treatment?

Phenoxybenzamine- irreversible non selective, alpha adrenergic blocker (longer duration of action than phentolamine)
Controls blood pressure and arrhythmia before surgery

485

Urethral opening on ventral shaft of penis is a result of malformation of what embryological structure?

Improper fusion of Urogenital folds- causes hypospadias

486

Noneveloped, single stranded RNA virus
Causes fulminant hepatitis in pregnant women
Spread via fecal-oral route, most commonly water-Bourne

Hepatitis E virus

487

Releases edema factor, acts by increasing intracellular cyclic adenosine monophosphate causing skin sores that are painless
What toxin also works by this mechanism?

^bacillus anthracis has edema factor

Vibrio cholera toxin and enterotoxigenic E.coli heat lability toxin creates similar effects, increasing cAMP.

488

Nausea, lack of appetite, sclera icterus, heptaomegaly and temporal wasting with elevated amylase, lipase, and alkaline phosphatase
Tumor marker CA19-9
Can have migratory thrombophlebitis, peripheral lymphadenopathy or pruritis and Courvoisier sign (painless, palpable GB)

Pancreatic adenocarcinoma

489

HNPCC, besides colon cancer, are at an increased risk for what other things?

Endometrial, ovarian, urinary tract, small intestine, stomach and biliary cancer

490

How are fats and fat soluble vitamins exit the digestive system to be absorbed into the body?

First enter lacteals, which are small lymphatic capillaries in the villi of the intestinal epithelium. Lacteals merge and the chyle flows until it reaches the thoracic duct, which drains into the left jugular venous angle.
Then stored in Ito cells in liver

491

Presents with elevated glucose, weight gain, impaired peripheral vision, sleep apnea, increased hand and foot size
Drug Treatment
What else is this treatment used for

Acromegaly- insulin resistance from GH stimulation, sleep apnea from macroglossia, impaired vision from a pituitary secreting tumor.
Treatment is octreotide- somatostatin analog
Secretory pancreatic tumors and carcinoid tumors

492

Hyperthyroid symptoms with no radioiodine uptake

Thyrotoxicosis factitia- taking exogenous Levothyroxine for weight loss
Increased T4 and T3 and low TSH

493

Child has failed to gain weight, lower than expected growth curves for both height and weight, frequent watery diarrhea, steatorrhea
Blunting of the intestinal villi and lymphocytic infiltration in lamina propria
Characteristically have what skin condition?

Celiac spruce with dermatitis herpetiformis- a erythematous, vesicular rash similar to herpes

494

Krukenburg tumor- metastatic tumor from what? Where else does this tumor spread?

Diffuse type gastric adenocarcinoma

Virchow node- left supraclavicular LN
(Thoracic duct drains alll structures on left in thoracic cavity and all structures below the diaphragm)

495

Traumatic injury in MVA, papilledema showing increased ICP, with acute stress ucler

Cushing ulcer- ICP stimulates vagal nucleus causing increased gastric acid secretion in stomach. Can result in superficial capillary bleeding

496

Cold thyroid nodule, fine needle aspiration finding of a psammoma body (nodular calcification)
“Orphan Annie nuclei” (ground glass appearance with central clearing)

Papillary thyroid carcinoma

497

Loud noises cause this muscle to contract and pull the tympanic membrane medially to allow transmission of sound
Muscle and innervation?

Trigeminal nerve (V3 mandibular division) innervates the tensor tympani
Close to middle meningeal artery
Damage leads to hypoacusis to low pitched sounds

498

Skip lesions with edematous cobblestone mucousa on colonoscopy, aphthous ulcers, intermittent diarrhea presentation with necrosis skin lesion and treatment

Chron’s disease with pyoderma gangrenosum- treat with oral corticosteroids

499

C. Diff toxin B mediates damage by:

Apoptosis

500

Fever that rises in the day and decreases at night, night sweats, weight loss, fatigue, and muscle and joint pain after previous good health.
History of travel to Mediterranean and dairy ingestion/contaminated livestock
Spreads via lymphatics and spleen- causes lymphadenopathy, hepatosplenomegaly, and strong moldy body odor.
Gram negative coccobacillus diagnosed by anti-LPS tiger

Brucellosis- infection with Brucella melitensis: gram negative coccobacillus which acid fast stains. Endemic to Mediterranean

501

Rapid onset of severe periumbilical abdominal pain ‘out of proportion of physical exam findings’
Peritoneal signs are generall absent until late in the disease process when infarction, leading to necrosis, and sometimes perforation has occurred.
Results from intestinal hypoperfusion
Chronically on histology showing irreversible cell injury- nuclear pyknosis, karyorrhexis, karyolysis

Acute ischemic bowel disease

502

Chronic diarrhea, weight loss, hyperglycemia unresponsive to oral hypoglycemic agents, and skin lesions characteristics of necrolytic migratory erythema, and a pancreatic mass on abdominal CT

Glucagonoma - neuroendocrine tumor from alpha cells of pancreatic islets

503

Erectile dysfunction medication and MOA
Contraindication:

Sildenafil- inhibits PDE-5 metabolism of cGMP, PDE-5 also inhibits NO induced vasodilation

Cannot be used with nitroglycerin

504

Long term Treatment of UC- antimetabolite prodrug that inhibits purine synthesis which is converted to?

Azathioprine—>6-mercaptopurine
Causes a reduction in number of WBCs= susceptibility to infection

505

Infant with history of cardiac surgery, recurrent infections, and cleft lip and palate, hypocalcemia
Microdeletion of 22q11- leads to?

Lack of development of 3rd and 4th pharyngeal pouches- give rise to thymus and parathyroid glands
DiGeorge syndrome

506

History of AIDs and fever, chest pain, dry cough with oval budding yeast ungulfed by macrophages
Treatment of which is what? AE?

Histoplasma capsulatum- dimorphic fungus in Midwest
Treat with ketoconazole - inhibits CYP450 enzymes in liver and must be monitored for liver dysfunction

507

Curling ulcer risk

Patients with severe burns can get ulcers- results in upper fundus of stomach and GI bleeds. From reduced plasma volume, which results in mucosal ischemia and necrosis.
Treat burn victims with omeprazole

508

Before surgery on pheochromocytoma patients, they must be administered what to lower their high blood pressure?
AE?

Irreversible alpha adrenergic antagonist
Miosis - vision in the dark will be affected

509

Analog that restores the actions of prostaglandins in the GI tract, resulting in reduced secretion of gastric acid and increased production and secretion of gastric mucous barrier to decrease NSAID induced PUD
AE of the drug-

Misoprostol
Causes uterine cramps and has potential abortifacent properties-labor induction

510

Normal flora of the vagina that combat infection

Lactobacilli- a gram positive facultative anaerobe

511

Treatment for diabetic gastroparesis - pro kinetic agent
AE- extrapyramidal symptoms
Acts as a muscarinic agonist in GI tract, causing increased LES tone, improved gastric peristalsis and emptying, and decreased pyloric sphincter tone.
D2 antagonist action at CNS also decreases nausea

Metoclopramide

512

Window period of Hepatitis B infection- about 2 months after presentation of symptoms as patient is improving. Serology?

Only anti-HBe and anti-HBc IgM are present. All others are negative
It is the period right before the development of anti-HBs antibodies in the recovery period

513

Low maternal alpha-feto protein, low estriol and low beta hCG on maternal serum triple marker screen during second trimester
Normal inhibin A

Edwards syndrome, trisomy 18- nondisjunction


(Trisomy 13- low hCG, others are normal)
(Down syndrome- low AFP and estriol, high hCG and inhibin A)

514

What is the primary reducing equivalent in the synthesis of steroids?
What is it derived from?

NADPH- only one that can be used in anabolic reactions like steroid synthesis
Derived from niacin- B3

515

Hepatitis B treatment in ESRD patients
Adenosine analog that inhibits reverse transcription and blocks hepatitis replication and inhibitions of viral replication

Entecavir and IFN alpha,resp.
Tenofovir more common in non ESRD patients

516

Very high hCG, US findings show lucent and echogenic areas with no fetal heartbeat detected
Triad- hyperemesis, vaginal bleeding and hyperthyroidism

Molar pregnancy- hydatidiform mole
Homology between hCG and TSH*

517

BPH treatment
Usually presents with urinary hesitancy, frequency, nocturia, or decreased stream
Enlarged prostate

Tamsulosin- alpha 1 adrenergic antagonist

518

First line treatment for acromegaly

Somatostatin analog- octreotide
Blocks the effect of GHRH to inhibit GH secretion
Inhibits gastric acid secretion and emptying
Inhibits insulin and glucagon secretion from pancreas

519

Neurophysiology I is essential for

Transport of oxytocin from paraventricular nucleus
Oxytocin facilitates milk secretion (prolactin facilitates milk synthesis)

520

Arthralgias, hypogonadism, lethargy, skin pigmentation and hepatomegaly
Also can have DM and dilated cardiomyopathy
Defect in the gene that encodes the HFE protein on (Chrom.) 6

Gene defect results in increase in efficacy of dietary iron absorption
This leads to increased hepatic storage- secretes more ferritin and transferrin becomes saturated with iron

Hemochromatosis

521

Drug that acts on PPARs to increase/act insulin receptors
Sensitive skeletal muscle and the liver to insulin, which decreases resistance and targets uptake of glucose
AE- water retention leading to heart failure with pulmonary edema and weight gain

Thiazolidinediones- piglitazone and rosiglitazone

522

what does testes dermining factor from SRY DIRECTLY affect?

Development of the testes from indifferent gonads.
The testes then secrete testosterone and AMH- give male phenotype from internal male genitalia

523

Nausea, vomit, abdominal pain radiating to his upper back and left shoulder and elevated lipase
This condition is usually assoc with what? How does it present?

Acute pancreatitis - often accompanied by hypocalcemia
Hypocalcemia- Chvostek sign: a muscle twitching following palpation of the cheek. QT prolongation

524

Complication of PID in 25% of cases
Presents as RUQ pain associated with the classic triad of PID- fever, abdominal pain and vaginal dischage

Perihepatitis

525

Presents with diarrhea, hypokalemia, and hypochlorhydria (low gastric acid) and pancreatic mass

VIPoma - secretes VIP. Normally secreted from parasympathetic ganglia in GI tract to cause secretion of water and electrolytes into lumen and relaxation of intestinal smooth muscle
Excessive VIP= inhibition of gastric acid secretion that leads to accumulation of chloride in the blood.

*WDHA- watery diarrhea, hypokalemia, achlorhydria

526

Amenorrhea, hypertension, impaired development of secondary sex characteristics and presence of a normal uterus and ovaries
Male patients- female phenotype at birth but lack internal female genitalia

17 alpha hydroxylase deficiency- rare CAH
Usually active in both adrenal gland and gonads catalyzing the steps in generation of corticosteroids and androgens
Does not play a role in mineralocorticoids
Impaired synthesis of cortisol, testosterone and estrogen
High ACTH that results from decreased cortisol stimulates the mineralocorticoid pathway leading to excessive corticosterone and DOC
DOC has similar affects as hyperaldosteronism- sodium reabsorption and potassium secretion

527

HLADR4 or 1+
Anti-CCP
RF

rheumatoid arthritis

528

Variant of a reed steenberg cell- lacunar cells: cytoplasm retraction. Appear separated from each other
Deposition of collagen- nodules
Mediastinal lymphadenopathy

Nodular sclerosing hodgkin’s

529

Anticoagulation for atrial fibrillation- long term: a direct factor Xa-i
Or direct thrombin inhibitor

Rivaroxaban, apixaban, edoxaban

Dabigatran

530

Synovial fluid- increased WBC, increased PMNs, high viscosity, normal glucose
Reaction to a GI or GU infection
Asymmetrical
+conjunctivitis, keratoderma, blenorrhagica on palms/soles, urethritis, circinate galant is on penis, and aortitis.

Reactive arthritis

531

Pheochromocytoma, medullary carcinoma of thyroid, parathyroid adenoma

MEN2A

2B- pheochromocytoma, medullary carcinoma of thyroid + mucosal neuroma, marfanoid body habitus

532

P450-I
Neurotoxicity by decreasing B6
Decreased synthesis of mycolic acids
Hepatotoxicty, SLE and anion gap metabolic acidosis

Isoniazid

533

Less than 5 yo
Viral surface contains fusion proteins (F protein), which causes membranes of nearby cells to merge, forming syncytial

RSV bronchiolitis- paramxyoviridae

534

Common carotids and proximal internal carotid are from which arch?

Third aortic arch

535

Consumes platelets and clotting factors
Risks- malignancy, sepsis or obstetric complications

DIC

536

Non Hodgkin lymphoma
Bcl-2 (anti-apoptosis gene) overexpression with IgH chromosome 14. T(14;18)
Middle aged patients
Wax/waning painless lymphadenopathy
B symptoms

Follicular lymphoma- follicular center B cells

537

Greater than 4.5 hours of ischemic stroke treat with :

Aspirin + warfarin

538

CAP treatment- in patient with extra risk factors vs previously healthy patients

Macrolide + 3rd generation cephalosporin or respiratory FQ (Levo/moxifloxacin)
Usually from S. Pneumonia
Healthy patients- macrolide or tetracycline

539

Oral flora of cats and dogs
Gram negative coccobacillus
Skin abscesses

Pastuerella multocida

540

Cell cycle nonspecific MOA
Alkylation agent that covalently bonds DNA
Very toxic

Cyclophosphamide

541

HTN in upper extremities and weak delayed pulse in lower extremities
Intercostal arteries enlarge and get notched appearance on ribs of chest X-ray
HF, risk of cerebral hemorrhage and bacterial endocarditis

Postductal coarctation

542

Paget’s labs and pathogenesis

Solitary increase in alkaline phosphatase
Dramatic remodeling increases bone mass
Decreased hearing: ossicles thicken
Thick trabeculae with mosaic pattern

543

Decreases the risk of GVHD
Treats autoimmune GN and RA
Interferes with purine synthesis. Liver metabolizes it to 6-mpu
Used for long term immunosuppression

Azathioprine

544

Hereditary CT disease of defective collagen type IV
Thin/split of glomerular BM and eye problems and sensorineural issues
Basket weave on EM

Alport Syndrome

545

All intrinsic laryngeal muscles innervated by recurrent laryngeal from what branchial arch?
Except 1 muscle innervated by:

Branchial arch 6

Cricothyroid muscle- superior laryngeal n from arch 4

546

Mylophosphorylase (Isozyme of glycogen phosphorylase) def in muscle. Normally free glucose-1-phosphate from glycogen in muscle
Muscle fatigue, rapid fatigue
Myoglobinuria

McArdle disease
Ischemic muscle test: forces to use glucose and lactate normally increases. Absent here

547

Innervates adductor muscles in medial thigh

Obturator nerve

548

Bronchoconstrictor:
Drugs Inhibits 5-lipooxygenase to block LT synthesis for asthma symptoms

(Bronchodilator equivalent)

Leukotriene C4
Zileuton

Prostaglandin E2

549

Alcoholics have decreased nutrition
Proline and lysine are not hydroxylated in endoplasmic reticulum
Bleeding gums, ecchymoses, petechiae, and corkscrew hairs

Scurvy: vitamin C deficiency

550

Eosinophils and their breakdown (Charcot Leyden crystals)
Hazy whorls of mucus (Curschmann spirals)

Bronchial asthma histology

551

Anion gap, metabolic acidosis and respiratory alkalosis (mixed disorder*)
Tinnitus

Aspirin toxicity

552

Trisomy 13
Midline defects of forebrain
Cleft palate, colobomas, micro and holoprosencephaly (failure of hemisphere division)
Intellectual disability, rockerbottom feet, polydactyly, congenital heart disease
Death by 1yo

Patau syndrome

553

Loss of one X chromosome
Short stature, webbed neck, flat/shield like chest, widely spaces nipples, lymphedema of hands and feet, wide angle elbows
Increased risk of coarctation of aorta- juxtaductal: distal to origin of subclavian a and opposite of ductus arteriosus
Can result in small left arm due to decreased blood flow if L subclavian involved
Bicuspid aortic valve

Turner syndrome

554

Bone pain, fractures, CRAB
Pancytopenia, Bence Jones proteinuria
Roleaux formation(hyper-viscosity)

Multiple myeloma

555

X linked recessive lysosomal storage disease: def of alpha galactosidase A
Accumulation of ceramide trihexoside in BV walls- angiokeratomas, Paresthesias, anhydrosis and heat intolerance
Chronically- renal, heart, or cerebral dysfunctional

Fabry disease

556

Loop diuretic used if sulfa allergy prevents furosemide usage, which is first line for CHF exacerbation

Ethacrynic acid

557

B symptoms
Binucleated cell with nucleoli- Reed sternburg cell
Treated with vincristine- microtubule inhibition

Hodgkin lymphoma

558

Altered mental status, fever, jaundice, anemia, thrombocytopenia, renal dysfunction, increased lactate and bilirubin
Schistocytes on blood smear
vWF protease ADAMTS13 is absent or decreased because of auto-AB or genetic mutation
Platelet adhesion without coagulation cascade activity
Will have normal PT/PTT

TTP (MAHA)

559

Colon distention : abdominal pain plus systemic toxicity signs (fever, tachycardia, anemia, dehydration, etc)
Double bubble sign
Can be secondary to antidiarrheals in kids less than 2 years old
Also from C.diff or UC

Toxic megacolon

560

Fatty stools
Cysts in stool and positive fecal ANTIGEN test
Increased risk in men who have sex with men, travel and unfiltered water

Giardia

561

Untreated hyperparathyroidism leads to overactive of osteoclasts, which increases serum calcium and alkaline phosphatase.
Decreased phosphorous for increased renal excretion
Recurrent microfractures and hemorrhages- ‘brown tumor’

Osteitis fibrosis cystica
In McCune-Albright syndrome

562

AE is restrictive lung disease
Inhibits DNA synthesis in G2 through free radicals that bind DNA and break strands
Used in testicular cancer and lymphoma

Bleomycin

563

Reticular formation induces REM
Ach changes during sleep/aging

Decreased Ach with age= decreased REM, total sleep time, and delta sleep
Seen in Alzheimer’s

564

Loeffler medium-
Chocolate agar-
Bordet-gengou agar-
Thayer- Martin agar-

Diphtheria
H. Flu
Bortedella pertussis
N. Gonorrhea

565

Migratory arthralgias of large joints, weight loss, diarrhea
Numerous vacuolated foamy macrophages in lamina propria

Whipple disease
Tropheyma whipplei infection

566

Intellectual disability, tall, thin, elongated limbs, scoliosis, subluxation of ocular lens
Cystathionine synthase and methionine synthase deficiency
Decrease cofactor B6
Thromboembolic episodes especially in brain

Homocystinuria

567

Lung cancer in superior primary sulcus growing into thoracic inlet
Can damage brachial plexus, stellate ganglion, sympathetic chain

Pancoast tumor

568

Cholestyramine
Use for

Binds negative charged bile acids, excreted in feces, then hepatocytes convert cholesterol to bile acid to replenish the loss, which decreases intracellular cholesterol concentration activity. Then Increase uptake of cholesterol containing LDL particles to decrease them in blood.

Familial hyperlipidemia

569

Gemfibrozil
MOA and AE

Fibrate used to decrease plasma triglycerides by stimulating LPL—> VLDL and chylomicrons to increase removal. Also increased HDL.
AE- myopathy and rhabdomyolysis

570

Radial nerve innervates

BEST
Brachioradialis, extensors of wrist and fingers, supinator, triceps
When damaged= wrists drop

571

Aorta supplies spinal cord in abdominal area. Major branch provides circulation to anterior spinal artery
Anterior spinal artery syndrome- affects all asc/desc pathways except the dorsal column (vibration and proprioception)

Artery of Adamkiewicz

572

Occupational exposure to heavy metals and paint chips
Basophilic stippling
Demyelination/degeneration of axons= peripheral neuropathy like wrist drop
Microcytic anemia- inhibits synthesis of heme at 2 levels: S-aminolevulinic acid DH and decrease iron incorporation
Acute intermittent porphyria: abdominal pain, psych, photosensitivity

Lead poisoning

573

Hypoalbuminemia, proteinuria, hyperlipidemia in kids
Occurs after a minor sinus immune insult
Loss of heparin sulfate proteoglycans: loss of filtering negative charge that usually prevents large anions from passing
EM- effaced food processes

Minimal change disease

574

Prophylaxis if CD4 less than 200 in HIV patients- trimethoprim sulfamethoxazole. If sulfa allergy: use aerosolized pentamidine.
Diffuse, interstitial pneumonia
Disc shaped yeast on methenamine silver stain

Pneumocystis jirovecii

575

Increased PT and PTT with normal platelets
Especially in infants of home births/lacked prenatal care
Controls gamma-carboxylation of glutamic acid on clotting factors II, VII, IX, and X + proteins C and S.

Vitamin K deficiency

576

Tumor at pyloric sphincter of lesser curvature
Impinges on omental foramen- formed by hepatoduodenal ligament: contains hepatic a proper, portal vein, and common bile duct
Bile duct can be obstructed which causes cholestasis and conjugated hyperbilirubinemia jaundice

Gastric adenocarcinoma

577

Peripheral blood smear of ruptured lymphocytes- smudge cells
Widespread lymphadenopathy
Autoimmune hemolytic anemia
Clonal B cells

CLL

578

HbS 35%-40% only
Tend to be symptom free except sickling in renal medulla- episodic hematuria from papillary necrosis and impaired ability to concentrate urine from microthrombembolic events brought on by high altitudes, pneumonia, physical exercise

Sickle cell trait

579

Parotitis- less time to modify saliva
Can be caused by clozapine, mumps, AI or bacteria
Normally reabsorb NaCl and secrete K and bicarb

Potassium remains static as flow rate increases- less secretion
Bicarb secretion increases independently
Increased rate is proportional to increase amylase

580

Kidney transporter and GI transporter of neutral AA is deficient
Tryptophan- essential precursor to niacin
Deficiency in niacin= pellagra: dev delay, abnormal gait, nystagmus, sun sensitive dermatitis, diarrhea

Hartnup disease

581

Chronic diarrhea, intermittent facial flushing, bronchospasm and tricuspid regurg
Major occur in midgut small bowel

Carcinoid tumor
Can cause syndrome if metastasized to the liver- otherwise liver will metastasize to 5-HIAA, which does not cause carcinoid

582

Chloride shift

Increased metabolic rate, more CO2 produced and enters RBCs, combined with water by CA to form bicarbonate acid—>H+ and bicarb. H+ buffered by deoxy Hb. bicarb diffuses out of RBCs in exchange for CL-

583

1-5 yo kid
Intermittent and colicky abdominal pain
Currant jelly stool and abdominal mass in RUQ
Commonly at ileocecal junction
Meckle diverticula and large mesenteric LNs are risk factors

Intussusception

584

Blocks release of NT at NMJ- Ach
Undergoes retrograde transport along axonal microtubules
Dunedin performs the retrograde transport by binding to microtubular tracks

Botulism toxin

585

Acute necrotizing vasculitis
High fever, congested, non-purulent conjunctivitis, change in lips/oral mucosa, rash, lymphadenitis
Coronary aneurysm is a complication

Kawasaki disease

586

Fatigue, muscle weakness, depressed mood, constipation
Commonly from antacids
Milk-alkali syndrome: BLANK, metabolic alkalosis, and renal insufficiency (low K and PO4)

Hypercalcemia

587

Starry sky granular IF
IgG, IgM and C3 deposition
Subepithelial humps
Type III HS

Post strep GN- group A strep pyrogens infection of pharynx or SKIN*

588

FEV1:FVC ratio is normal but both are low
Amiodarone is an anti-arrhythmic that can cause this AE

Restrictive lung disease- pulmonary fibrosis and interstitial pneumonitis

589

Iron deficiency anemia (low MCV, low MCHC, low RBC, high RDW)
Esophageal web
Atrophic glossitis- atrophy of filiform papillae in long standing iron deficiency

Plummer Vinson syndrome

590

Megaloblastic anemia with increased levels of methylmalonic acid and homocysteine
Often presented with GI problems that reduce absorption in ileum
Hypersegmented neutrophils
Enzymes affected: methionine synthase and methylmalonyl CoA

Vit B12 deficiency

591

Hogkin’s lymphoma treatment- intercalates and inhibits DNA replication
Can cause cardiomyopathy- dilation of all 4 chambers
Leads to systolic dysfunction with decreased EF, narrow pulse pressure and jugular venous distention
Dilated cardiomyopathy-S3 heart sound and systolic regurg murmur

Doxorubicin

592

Calcification of valve cusps from normal aging or congenital bicuspid valve in younger patients
Crescendo-decrescendo and systolic ejection click
Increased LV pressure in systole
Radiates to carotids
Syncope, angina, dyspnea on exertion

Aortic stenosis

593

Acute onset of tremor and disorientation, increased HR/BP
Peaks 2-5 days after patient’s last drink
Treatment: chlordiazepoxide (benzo) and lorazepam if have liver disease

Delirium tremens from alcohol withdrawal

594

T(9;22) Philadelphia chromosome- constitutively act. TK from the bar-abl translocation
Increased granulocytes- mostly neutrophils
Basophila
Can accelerate to a blast crisis: AML or ALL
Older patients
Rx- imatinib: TK-inhibitors

CML

595

Centrally located lung tumor associated with smoking
Paraneoplastic syndrome- SIADH, ACTH, antibodies against calcium presynaptic receptors: Lambert Eaton
Neuroendocrine Kulchinsky cells- small dark blue cells
Chromogranin A+

Small cell lung cancer

596

Inhibits neuronal uptake of NE and serotonin in CNS
AE: sedation, postural hypotension, anticholingeric effects, increased QT, arrhythmia, respiratory depression, hyperpyrexia, confusion, hallucinations
**TdP: treat with Mg2+

TCAs- amitriptyline and imipramine

597

Direct acting sympathomimetics- bind post synaptic adrenergic receptors, more selective receptor activity, do not go into CNS, produce an increased bronchodilator response, short acting, quick onset, decreased BA
Indirect acting sympathomimetics- catecholamine release via presynaptic terminals, less selective, go into CNS, last longer, increased BA

Direct- NE/Epi, DA

Indirect- amphetamines, cocaine, ephedrine

598

Gram negative diplococci
Second most common cause of meningitis in kids- septicemia, fever, tender neck, maculopapular rash and petechiae on trunk
Can cause Waterhouse Friderichsen syndrome: sepsis with adrenal insufficiency
CSF- bacterial meningitis: high WBC, low glucose, increase proteins

Neisseria meningitidis

599

Fever, Lethargy, a new heart murmur
Roth Spots: retinal hemorrhages
Osler nodes: painful, erythematous lesions on hands/feet
Splinter hemorrhages: tiny blood clots under the nail

Acute Bacterial Endocarditis

600

Increased lung volumes causes alveolar expansion, causing _______ of the alveolar BVs, which has what effect on alveolar vessel resistance?
What is the affect when lung volumes decrease?
How do you calculate PVR? Where is it lowest?

Stretching of the vessels, causing a decrease in diameter, which increases the alveolar vessel resistance

decreased volumes cause vessels to become narrowed also, due to the compression by positive intrathoracic pressure, which leads to increased extra-alveolar resistance

PVR is a sum of alveolar and extra-alveolar resistances
Total PVR is a U curve, with the lowest near the FRC (end of a normal expiration)

601

______ nerve arises from L1, emerges from the lateral border of the upper psoas major and passes behind the kidney. It’s superficial branch emerges above the superficial ring to innervate the skin above the pubic region
Can be injured during what surgical procedure?

Which nerve arises from L1 and accompanies the spermatic cord through the superficial ring to provide sensation to the upper and medial thigh and parts of external genitalia?

Iliohyopgastric- can be damaged during appendectomy

Ilioinguinal

602

What is tagged on an immunoflourescent microscopy of a glomerulus that has undergone crescent formation?

Fibrin*

603

Neonate born prematurely and have severe respiratory distress is given oxygen supplementation. What findings is this likely to cause in the retina?

Abnormal retinal vascularization that extends into vitreous
Local hyperoxia induces VEGF upon return to room air ventilation

604

What two labs should be monitored with lithium administration

BUN/creatinine for renal function (causes nephrogenic DI)
TSH (hypothyroidism AE)