NBME 12 - 5/28/2014 Flashcards
Mousy body order think?
Phenylketonuria
What is the pathobiology of PKU. Inheritance? Tx? Diatary restrictions?
A/R. Screened for 2-3 days after birth (normal at birth because of maternal enzyme during fetal life). Decreased phenylalanine hydroxylase or decreased tetrahydrobiopterin cofactor (malignant PKU). Tyrsoine becomes essential. Increased phenyalanines leads to phenylketons in urine. Findings: intellectual disability, growth retardation, seizures, fair skin, eczema, musty body odor. Tx: decreased phenylalanine and increased tyrosine. Avoid artificial sweetener like aspartame that has phenylalanine
What are the retroperitoneal structures?
SAD PUCKER - suprarenal glands (adrenals), aorta and ivc, Duodenum (2nd through 4th), pancreas except tail, uterus, colon (descending and ascending), kidneys, esophagus (lower 2/3), rectum (partial)
Treat ehrlichiosus with? MOA
Doxycycline - binds to 30s and prevents attachment of aminoacyl-tRNA
What protein is upregulated on endothelial cells for loose binding of neutrophils?
E-selectin and P-selectin
What is hereditary spherocytosis? What does it look like on histology? Lab tests?
Histology: spherocytes - small(er) cells with no central clearing. Defect in proteins interaction with RBC plasma membrane and skeleton - (ankryin, band). Less membrane causes cell to be smaller and rounder with no central pallow (increased MCHC and red cell distribution width) - premature removal of spleen. Can find intermittent jaundcie, splenomegaly and aplastic criss with parvovirus B19 infection. osmotic fragility test
Do what kind of analysis to dx klinefelters?
Chromosomal analysis of lymphocytes (easy access)
Diverticulosis is a/w? What is it?
Low fiber diets. Many false diverticular of the colon, commonly sigmoid. Common in 50% of ppl over 60. caused by increased intraluminal pressure and weakness in colonic wall. a/w low fiber diets
What is the most common cause of septic arthritis in sexually active young people? How do you treat it?
N Gonorrhea. Ceftriaxone (and add doxycycline for possible chlamydia infection)
Know what CMV infection looks like on histology! Infection with CMV means deficiency in what kind of cells?
T lymphocytes
Portan hypertension causes - Portovacal anastomses for 1) eosphageal varices 2) caput medusae 3) hemorrhaids
1) portal: left gastric // systemic: esophageal 2) portal:paraumbilical // systemic: superficial and inferior epigastric 3) portal: superior rectal // systemic: inferior and middle rectal
Dysfunction of what protein allows neoplastic cells to detach from primary tumor and advance through adjacent BM?
E- cadhedrin
Dopamine agonist drug?
Bromocriptine
What is the MOA for pioglitazone?
Increased insulin sensitivity in peripheral tissue. Binds to PPAR-gamma nuclear transcription regulator.
With chronic neuropathy, regenerating axon will do what?
Normally you have type 1 and 2 muscles fibers evenly distributed in a muscle biopsy. Let’s say you have a disease like ALS where you have death of peripheral neurons. The body tries to compensate via axonal regeneration. The regenerating axon will then innervate the muscle fibers in a given area of deinnervation. All muscle fibers will assume either type 1 or type 2 depending on what type of muscle the neuron originally innervated. On biopsy you no longer have a checkboard appearance of type 1 and 2 fibers. They’ll either be pure type 1 or pure type 2.
