6/6 Mixed

Question 1

Standard deviation

Answer 1

mean +/- 1.96*SD would cover 95% of the observations

Mean +/- 2.58*SD would cover 99% of the observations

Question 2

Hyperimmunoglobulin M (hyper-IgM)

Answer 2

Defective immunoglobulin class switching due to a defect in CD40 ligand-CD40 interaction
Absence of CD40 ligand is the most common cause and is inherited in an X-linked recessive pattern
Recurrent sinopulmonary, GI, and opportunistic infections

Question 3

Mycoplasma antibiotic resistance

Answer 3

All organisms in the mycoplasma genus lack peptidoglycan cell walls and therefore resistant to agents that attack the peptidoglycan cell wall such as penicillins, cephalosporins, carbapenems, and vancomycin
Rx with anti-ribosomal agents (tetracycline, macrolides)

Question 4

Age 3 development

Answer 4

Play imaginatively in parallel, speak in simple sentences (3 words), copy a CIRCLE, use utensils, and ride a tricycle

Question 5

CFTR protein

Answer 5

chromosome 7

transmembrane ATP-gated chloride channel

Question 6

Location of left kidney

Answer 6

Lies immediately deep to the tip of the 12th rib

Question 7

Luekomoid reaction

Answer 7

Benign leukocytosis (>50,000) that occurs in response to an underlying condition such as severe infection/hemorrhage, malignancy (Leukemia) or acute hemolysis
Leukocyte alkaline phosphatase levels are normal or increased
Peripheral smear can show increased bands, early mature neutrophil precursors (myelocytes) and granules (Dohle bodies) in the neutrophils “basophilic oval inclusions in mature neutrophils”

Question 8

Glioblastoma

Answer 8

Most common primary cerebral neoplasm of adults that is typically located within the cerebral hemispheres and may cross the midline
These tumors are highly malignant and grossly contains areas of necrosis and hemorrhage

Question 9

First and zero order kinetics

Answer 9

In first order kinetics, a constant FRACTION (or proportion) of drug is metabolized per unit of time, so the amount metabolized changes based on the serum concentration

In zero-order kinetics, a constant amount of drug is metabolized per unit time, independent of serum levels

Question 10

Hypertrophic cardiomyopathy

Answer 10

Dynamic left ventricular outflow tract obstruction is due to abnormal systolic anterior motion of the anterior leaflet of the mitral valve toward a hypertrophied interventricular septum

Question 11

Gaucher disease

Answer 11

AR, lysosomal storage disorder that is characterized by B-glucocerebrosidase deficiency and presents with pancytopenia and hepatosplenomegaly
Glucocerebroside accumulation leads to distended macrophages with a “wrinkled tissue paper” appearance, also known as Gaucher cells

Question 12

Renal artery stenosis

Answer 12

Causes renal hypoperfusion and activation of the RAAS
Modified smooth muscle (juxtaglomerular) cells of the afferent glomerular arterioles synthesize renin
This leads to a cascade of effects that include systemic vasoconstriction (ATII) and sodium and water retention (aldosterone and ATII)
Chronic renal hypoperfusion will cause hypertrophy and hyperplasia of the juxtaglomerular cells

Question 13

Communicating hydrocephalus

Answer 13

Symmetrical enlargement of the ventriculi
Usually occurs secondary to dysfunction or obliteration of subarachnoid villi
This is usually a sequelae of meningeal infection (including tuberculosis meningitis) or subarachnoid/intraventricular hemorrhage)

Question 14

Acute hepatitis A

Answer 14

Self-limited infection that typically presents acutely with prodromal symptoms (fever, malaise, anorexia, N/V, right upper quadrant pain) followed by signs of cholestasis (jaundice, dark-colored urine, clay-colored stool)
Livery biopsy: spotty hepatocyte necrosis and inflammatory cell infiltration

Question 15

Alpha 1 receptor stimulation of eye

Answer 15

Causes contraction of the ocular pupillary dilator muscle, resulting in mydriasis (pupillary dilation)

Question 16

Locating the appendix during surgery

Answer 16

The teniae coli are 3 separate smooth muscle ribbons that travel longitudinally on the outside of the colon and converge at the root of the vermiform appendix
If the appendix cannot be identified by palpation during an appendectomy, it can be located by following the teniae coli their origin at the cecal base

Question 17

Cholesteatomas

Answer 17

Collections of SQUAMOUS CELL DEBRIS that arise from a mass behind the tympanic membrane
Can be congenital or may occur as an acquired primary lesion or following infection, trauma, or surgery of the middle ear
They can cause hearing loss due to erosion into auditory ossicles

Question 18

Digoxin toxicity

Answer 18

Typically presents with cardiac arrhythmias and nonspecific GI (N/V), neurological (confusion, weakness), and visual symptoms
Elevated K is another sign and is caused by inhibition of the Na-K-ATPase

Question 19

HIV-associated dementia

Answer 19

Histopathologic findings: microglial nodules, groups of activated macrophages/microgial cells formed around small areas of necrosis that may fuse to form multinucleated giant cells

Question 20

Narcolepsy

Answer 20

Recurrent lapses into sleep or napping multiple times within the same day, occurring at least 3 times weekly for 3 months with at least 1 of the following:

1. Cataplexy (conscious, brief episodes of sudden bilateral muscle tone loss precipitated by emotions of laughter or joking, or spontaneous abnormal facial movements without emotional triggers)
2. Hypocretin-1 deficiency by CSF analysis
3. Rapid eye movement sleep latency

Question 21

Chlamydia trachomatis serotype L1 through L3

Answer 21

Cause lymphogranuloma venereum
Sexually transmitted disease characterized initially by painless ulcers with later progression to painful inguinal lymphadenopathy (“buboes”) and ulceration
Clamydial inclusion bodies are seen in host cell cytoplasm

Question 22

Adenomatous polyps

Answer 22

Contain dysplastic mucosa and are premalignant
Regular screening with timely excision of polyps is effective for prevention of colon adenocarcinoma
Increased activity of cyclooxygenase 2 linked to some forms of colon adenocarcinoma
Regular aspirin use decreases adenomatous polyp formation

Question 23

Tryptase

Answer 23

Specific to mast cells and can be used as a marker for mast cell activation (anaphylaxis)

Question 24

Tumor marker for Hepatocellular carcinoma and germ cell tumors

Answer 24

Alpha-fetoprotein

Question 25

Ankylosing spondylitis

Answer 25

Limits chest wall expansion, leading to hypoventilation

Question 26

Henoch-Schonlein Purpura (HSP)

Answer 26

Small vessel leukocytoclastic angiitis associated with IgA and C3 deposition, children 3 to 11, related to recent infection, palpable skin lesions, with or without abdominal pain and arthralgias
can develop glomerulonephritis and ESRD

Question 27

Cystinuria

Answer 27

Hexagonal cystine crystals