test #21 4.11 Flashcards Preview

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Flashcards in test #21 4.11 Deck (114)
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1

tumors associated w/ von-Hippel Lindau

hemangioblastomas, pheochromocytoma, and renal cell carcinoma

2

mutations in retinoblastoma associated with..

osteosarcoma and bilateral retinoblastoma (Rb - tumor suppressor involved in transition of G1 to S)

3

location of MEN1 mutation

MENIN gene chromosome 11

4

which MEN syndrome is associated with marfanoid habitus

MEN2B

5

sign of mature fetal lungs in amniocentesis

lecithin / sphingomyelin ratio > or = 2.

lecithin = phoshatidylcholine.
mature = making surfactant

6

in suspected erythroblastosis fetalis, measure amniotic fluid for..

billirubin

7

amniotic measurement of 17-hydroxyprogesterone tests for..

21-B hydroxlase deficiency

8

main form of therapy for urea cycle (ornithine cycle) defects?

protein restriction (enough for growth & development though)

9

heterozygotes for sickle cell trait (HbAS) are..

protected from sickle cell crises, aplastic crises, sequestration crises (bc >50% HbA).

protection from Plasmodium falciparum.

peripheral smears are usu normal, as is retic count.

WILL sickle when sodium metabisulfite is added

10

increased MCHC

mean corpuscular hemoglobin concentration. increased when dehydrated.

11

nasal mucosal ulcerations & glomerulonephritis

wegener's (granulomatosis w/ polyangiitis)

12

c-ANCA + is virtually pathognomonic for..

Wegener's (granulomatosis w/ polyangiitis). target neutrophil proteinase 3

13

smooth muscle cell antibodies..

autoimmune hepatitis

14

antimitochondrial antibodies

primary biliary cirrhosis

15

expectations by age 3

parallel play (not cooperative), tricycle, climb stairs, identify self w/ name and gender, copy shapes.

16

most common cause of aseptic meningitis?

eneterovirus (90%) of cases. fecal-oral (coxsackie, echo, polio) replicate in GI tract but usu don't cause gastroenteritis.

17

presentation of poliomyelitis

unvaccinated immigrant from endemic regions.

fever, malaise, aseptic meningitis first ((photophobia, painful extraocular muscle movements)

then myalgia and asymmetric paralysis (classically affecting legs) -- damage to lower motor neurons -- HYPOREFLEXIC paralysis

18

physiostigmine

rx: atropine overdose. tertiary amine, so crosses blood brain barrier (fixes PNS and CNS). acetylcholinesterase inhibitor

19

which artery runs with radian nerve?

DEEP brachial artery.

brachial artery travels medially along arm into antecubital fossa.

20

midshaft fracture of humerus (2)

radian n. & deep brachial artery damage

21

non-extensor actions of radian n. (2)

brachioradiali (forearm flexor) also supinator muscles

22

supracondylar fractures of humerus (2)

proximal median n. & brachial artery

23

periodic acid-schiff reaction

periodic acid oxidizes carbon-carbon bonds --> forms aldehydes that product magenta when react w/ fuschin-sulfurous acid.

good at detecting polysaccharides, mucosubstances, basement membranes.

diastatse + PAS = glycogen (will digest glycogen to maltose and glucose, which will wash off, negative rxn)

24

strongly PAS+ intestine

whipple disease (infxn w. tropheryma whipplei)

25

describe malabsorption & symptoms of whipple disease

infection w/ the actinomycete tropheryma whipplei in macrophages of small intestine--> foamy macrophages --> compress lacteals and impair fat absorption. PAS+ & diastase-resistant intestine.

foamy whipped -- CANN
cardiac, arthralgia, mesenteric nodes, neurological symptoms.

usu older men.

26

calcium colour on H&E

dark purple

27

iron stain

prussian blue, dark blue

28

neutral lipid stain

nile red or sudan black

29

path of CN III

courses between PCA and SCA as it leaves midbrain. susceptible to injury from aneurysm in these vessels.

travels w/ PComm.

30

aneurysms in PComm, PCA, or SCA can affect

oculomotor n.