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Flashcards in 4/9 Biochem Deck (182)
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1

Which enzyme is absent in Pompe's disease?
-does it present w/hypoglycemia?

alpha-1,4-glucosidase (acid maltase).
-no it does not present w/hypoglycemia.

2

Fragile X syndrome
-defect affecting the ______ & expression of the _____ gene.
-inheritance pattern?

-methylation, FMR1.
-x-linked recessive.

3

Are Power and sample size related?

Yes, directly related.
-inc. sample size leads to inc. power of a study.

4

Why does hyponatremia (ie. from thiazides) lead to lithium toxicity?

B/c PCT will then try to resorb more Na and will resorb lithium as well bc of the similar structure. So you'll be resorbing too much lithium.
-lithium has a narrow therapeutic index.

5

If splenic art is blocked, which arteries that come off of it have poor anastomoses?

short gastrics.

6

CREST syndrome
-what does the T stand for?
-what are the main Sxs of esophageal dysmotility?
-what will barium study show of esophagus?

-telangiectasis!
-heartburn, regurg, dysphagia.
-dilated esophagus & absent parastalsis

7

"corkscrew esophagus" seen in what disease?

diffuse esophageal spasm

8

CREST syndrome
-mechanism behind esophageal dysmotility?

fibrous replacement of muscularis.

9

Coronary steal syndrome
-what is the key?
-most often seen w/which drugs?

Key is that collaterals will form distal to the block, and the tissue fed by the blocked areas will be releasing vasodilators due to the ischemia.
-if you give a coronary vasodilator, now the other cornaries (in healthy parts) are also vasodilated, so more blood shunted to them and less blood to ischemic areas!

*seen w/coronary artery vasodilators - adenosine, dipyridamole.

10

Selective coronary artery vasodilators: name 2:

adenosine, dipyridamole.

11

How many hours of fasting can glycogen stores last before they're depleted?

12 to 18 hours.
-then gluconeo takes over.

12

Viral bronchiolitis
-whats the most common cause?
-Tx?
-Tx for prevention?

RSV
-ribavirin.
-palivizumab.

13

Phenytoin and valproate inhibit intestinal absorption of which vitamin?
-can lead to what birth defects?

B9 (folate)
-can lead to neural tube defects.

14

PDA
-deriv of which aortic arch?

6th aortic arch.

15

Pericardial knock
-seen in what?
-what is it?

-constrictive pericarditis.
-ventricular compliance reduced via an external force.

16

Neutrophil rolling:
-endothelial cells display what?
-neutros display what?

-selectins
-sialyl lewis acid

17

Hashimotos
-what will you see histologically?

mononuclear, parenchymal infiltration w/well-developed germinal centers.

18

Infection, pain, sleep deprivation.
-what will blood glucose levels be? inc or dec?
-therefore, will these precipitate a hypoglycemic state in a diabetic?

increased
-typically due to catecholamine release.
-no they will not.

19

Severe hypoglycemia
-Tx:

intramuscular glucagon.
-will stimulate glycogenolysis & gluconeo.
-pt will return to consciousness in 10-15 min.

20

Purely ketogenic AAs:
-mnemonic:

Lysine and Leucine
-the onLy pureLy ketogenic amino acids.

-Ketogenic = producing acetoacetate or its precursors.
-Glucogenic = producing TCA intermediates or pyruvate

21

Inc. serum alanine starting in infancy.
-which disease?
-Tx:

PDH deficiency.
-inc. intake of ketogenic nutrients (e.g., high fat content or inc. lysine and leucine).

22

Malate to OAA
-produces what?
-what happens in alcoholics?

-Malate to OAA = make NADH. So in alcoholics w/high NADH, opposite rx takes place, OAA => malate, producing NAD+.
-since you need OAA for gluconeo, this inhibits gluconeo, which is why alcoholics get resting hypoglycemia.

23

Tender loving care for nancy enzymes:

PDH, alpha-KG-DH, branched chain DH.
*these all req thiamine.
*transketolase ONLY req thiamine.

24

Substrate level phosphorylation steps:

-PEP
-1,3-bisphosphoglycerate
-succinyl-coa.

25

Rotenone, cyanide, antimycin A, CO.
-how do they poison us?

Electron transport inhibitors.

26

Oligomycin.
-how does it poison us?

ATP synthase inhibitor.

27

Uncoupling agents
-name some:

-2,4-Dinitrophenol (used illicitly for weight loss).
-aspirin (fevers often occur after aspirin overdose).
-thermogenin in brown fat.

28

Electron transport chain
-protons pumped into which part of the mito?
-where is the ADP made into ATP?

-intermembrane space.
-ATP made in mito matrix, the ATP synthase is in inner-mito membrane.

29

Glucose-6-phosphatase
-where is this enzyme found?

Liver
-also kidney & intestinal epithelium.

30

glutathione
-cofactor for which enzyme?
-action of enzyme?

-glutathione peroxidase which prevents oxidative damage by converting H2O2 → H2O.
*This is especially important in RBCs.