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Flashcards in Keyword associations Deck (42):
1

aschoff bodies

necrotic granulomatous inflammation seen in rheumatic fever in heart

2

lysyl hydroxylase

defective collagen fibril cross linking--Ehler Danlos

3

FGF3 receptor defect

achondroplasia (dwarfism)

4

lysosomal α-1-4-glucosidase defect

Pompe's disease

5

carbonic anhydrase II

osteoclasts cannot properly absorb bone -->osteopetrosis

6

hexosaminidase A defect

Tay Sachs--accumulation of GM2 ganglioside

7

sphingomyelinase defect

Niemann Pick--accumulation of sphingomyelin

8

B-glucocerebrosidase defect

Gaucher's disease--accumulation of glucocerebroside

9

alpha-galactosidase a defect

Fabry's disease-- ceramide trihexoside

10

galactocerebrosidase defect

Krabbe's Disease

11

chromosome 17 tumor supressor gene

p53--Li Fraumeni syndrome

12

iris speckling (brushfield spots)

Down's Syndrome

13

blurry yellow vision

digoxin toxicity

14

brown stain with toluidine blue

metachromatic lipid cx of metachromatic leukodsystrophy; arylsulfatase A deficiency; accumulation of cerebrosidase sulfate

15

right-sided heart pathology in setting of diarrhea

carcinoid syndrome

16

hypersalivation, nystagmus, and tachycardia

PCP intoxication

17

statins + fibrates

increased risk of myositis and rhabdomyolysis

18

azathioprine + allopurinol

aplastic anemia, fatal hematologic disorders: azathiprine is metabolized to 6-MP which is inactivated by xanthine oxidase (but is inhibited by allopurinol). toxic 6-MP buildup

19

coarse facial features, diffuse joint stiffness, claw hand deformities, and kyphoscoliosis. Muscle biopsy reveals numerous intracytoplasmic inclusions in cells

I cell disease

20

Disulfiram-like drug reaction

disulfiram-like reactions include metronidazole, griseofulvin, chloramphenicol, chlorpropamide, and some cephalosporins including cefamandole and cefoperazone, tolbutamide

21

Good mnemonic for muscle sarcomere bands

An Interesting Zoo Must Have Mammals: Actin in the I-band attached at the Z-line. Myosin in the H-band attaches at the M-line.

22

neurophysin point mutation

Carrier protein that binds to ADH and oxytocin in posterior pituitary. Can lead to autosomal dominant hypothalamic diabetes inspidus.

23

PAS + intestinal biopsy

stains glycoprotein of tropheryma whipplei brilliant pink (diastase negative) --Whipple's Disease

24

craniopharyngioma

calcified cystic mass, remnant of Rathke's pouch, pituitary gland derivative, cholesterol filled

25

euvolemic hyponatremia

SIADH

26

chronic lymphedema, hard violaceous cutaneous nodules

Angiosarcoma, Stewart-Treves syndrome

27

What drug to avoid in patient with BPH and depression?

TCAs--like amitryptiline have anticholinergic SEs that may worsen urinary retention.

28

chromosomal instability disorders

xeroderma pigmentosum, Fanconi anemia, Bloom's syndrome, ataxia-telangectasia

29

hypermethylation disorders

Fragile X syndrome (hypermethylation, CGG repeats, FRM1 gene)

30

mismatch repair defect

Lynch syndrome (HNPCC)

31

jawpain with meal, tongue pain

temporal arteritis--get ESR

32

Waterhouse friedrichson syndrome

adrenal hemorrhage from meningococcal septicemia; hyponatremia, hyperkalemia, hypoglycemia

33

VIPoma

WDHA syndrome (watery diarrhea, hypokalemia, achlorhydria)

34

valproic acid in pregnancy

folate absorption inhibition, neural tube defects

35

pure RBC aplasia

parvo B19, thymoma, lymphocytic leukemia

36

non-cytotoxic Type 2 hypersensitivity reactions

4G's: Graves disease, Gravis myasthenia, Gastric (pernicious) anemia, and Genetic (type II) diabetes

37

type 4 hypersensitivity reactions

Guillain Hashimoto Contacts Multiple PPD+ Type I Diabetic 4 a Graft: Guillain Barre Syndrome; Hashimoto thyroiditis; Contact Dermatitis; Multiple Sclerosis; PPD test; Type I Diabetes; Graft vs. Host disease

38

drugs that cause drug induced SLE

hydralazine, procainamide, isoniazid, minocycline, quinidine

39

drug used to diagnose prinzmetal's angina

ergonovine test; ergot alkaloid that stimulates alpha/dopa/serotonergic receptors-->vasoconstriction

40

destruction of bilateral adrenal glands, DIC, shock

Waterhouse Friedrichson, N meningitidis bacteremia

41

appetite suppressants: life threatening side effect?

RVH--fenfluramine, phentermine

42

poly ribosyl ribitol phosphate antibodies

immunity against Hib