Dit Day 13

Front 1

rate-determining enzyme of heme synthesis

Back 1

aminolevulinate synthase

Front 2

rate-determing enzyme of bile acid synthesis

Back 2

7 alpha hydroxylase

Front 3

which immunosuppressant matches the following statement?

precursor of 6-mercaptopurine

Back 3

azathioprine

Front 4

which immunosuppressant matches the following statement?

may prevent nephrotoxicity with mannitol diuresis

Back 4

cyclosporine

Front 5

which immunosuppressant matches the following statement?

antibody that binds to CD3 on T cells

Back 5

muromonab

Front 6

which immunosuppressant matches the following statement?

antibody that binds IL-2 receptor on activated T cells

Back 6

daclizumab

Front 7

which immunosuppressant matches the following statement?

inhibits inosine monophosphate dehydrogenase

Back 7

mycophenolate

Front 8

which immunosuppressant matches the following statement?

inhibits calcineurin --> loss of IL-2 production --> blockage of T cell differentiation and activation

Back 8

cyclosporine

Front 9

which immunosuppressant matches the following statement?

binds FK-binding protein --> loss of IL-2 production

Back 9

tacrolimus

Front 10

which immunosuppressant matches the following statement?

binds FKBP-12 --> inhibition of mTOR --> inhibition of T cell production

Back 10

sirolimus

Front 11

which immunosuppressant matches the following statement?

used for lupus nephritis

Back 11

mycophenolate

Front 12

which immunosuppressant matches the following statement?

metabolized by xanthine oxidase, therefore allopurinol increases its toxicity

Back 12

azathioprine

Front 13

what pathology matches the following statement?

1) pigemented hamartomas in the iris
2) owl's eye inclusions
3) owl's eye protozoan

Back 13

1) lisch nodules in NF type 1
2) CMV
3) giardia lamblia

Front 14

what histological change takes place in the trachea of a smoker?

Back 14

ciliated columnar cells --> squamous metaplasia

Front 15

the following lung volumes are obtained from an elderly smoker: FRC 5.0L, IRV 1.5 L, IC 2.0L, VC 3.5L --> what is his total lung capacity?

Back 15

7.0 L (FRC+IC = TLC)

Front 16

what is the genetic abnormality in primary pulmonary HTN?

Back 16

BMPR2 gene (bone morphogenetic protein receptor type II)

Front 17

what is the ddx for eosinophilia?

Back 17

"D NAAACP"

drugs
neoplasm
allergy, asthma (Churg-strauss)
Addison's (adrenal insufficiency)
AIN (acute interstitial nephritis)
collagen vascular dz
parasites (Loffler's eosinophilic pneumonitis due to Ascaris lumbricoides)

Front 18

a pt presents w/ asthma attack --> what immunological rxn is taking place that is responsible for anaphylaxis in this pt?

Back 18

antigen crosslinking IgE on mast cells, leading to histamine release

Front 19

most common cause of PNA in children 1 y/o or younger

Back 19

RSV

Front 20

MCC of PNA in neonate (birth - 28 d)

Back 20

GBS, E. coli

Front 21

a 30 y/o comatose man on ventilatory support in the ICU develops infxn and dies. autopsy reveals a pus-filled cavity in right lung --> what is most likely etiology?

Back 21

aspiration --> lung abscess

Front 22

tx of pulmonary HTN?

Back 22

bosentan (main one)
prostaglandin, sildenafil, DHP-CCB (nifedipine)

Front 23

what drug class is fexofenadine?

Back 23

2nd gen h1 blockers (less sedating and less antimuscarinic effects, better for old people with h/o falls)

Front 24

which lung CA type is a/w hypercalcemia?

Back 24

squamous cell carcinoma (b/c it is a/w PTHrP)

Front 25

what is the name of the genetic syndrome:
alcoholics --> B1 deficiency --> neurologic defects

Back 25

pyruvate DH deficiency

Front 26

what is the name of the genetic syndrome:

deficiency of aldolase B

Back 26

fructose intolerance

Front 27

what is the name of the genetic syndrome:

deficiency of cystathionine synthase

Back 27

homocysteinuria

Front 28

what is the name of the genetic syndrome:

galactose-1-P uridyltransferase deficiency --> MR, HSM, cataracts

Back 28

galactosemia

Front 29

what is the name of the genetic syndrome:

deficiency of tyrosinase

Back 29

albinism

Front 30

what is the name of the genetic syndrome:

inherited defect in tubular amino acid transporter

Back 30

cystinuria

Front 31

what is the name of the genetic syndrome:

deficiency in homogentisic acid oxidase

Back 31

alkaptouria

Front 32

what is the name of the genetic syndrome:

hypoglycemia + jaundice + cirrhosis

Back 32

fructose intolerance

Front 33

what is the name of the genetic syndrome:

rx = acetazolamide to alkalinize urine

Back 33

cysteinuria

Front 34

what is the name of the genetic syndrome:

rx = decreased fructose and sucrose intake

Back 34

fructose intolerance

Front 35

what is the name of the genetic syndrome:

rx = increased intake of ketogenic nutrients (fats)

Back 35

pyruvate DH deficiency

Front 36

what amino acid is a precursor to the following molecule?

histamine

Back 36

histadine

Front 37

what amino acid is a precursor to the following molecule?

porphyrin, heme

Back 37

glycine

Front 38

what amino acid is a precursor to the following molecule?

NO

Back 38

arginine

Front 39

what amino acid is a precursor to the following molecule?

GABA

Back 39

glutamate (via glutamate decarboxylase)

Front 40

what amino acid is a precursor to the following molecule?

S-adenosyl-methionine (SAM)

Back 40

methionine

Front 41

what amino acid is a precursor to the following molecule?

creatine

Back 41

arginine

Front 42

what amino acid is a precursor to the following molecule?

1) urea
2) glutathione
3) serotonin, melatonin

Back 42

1) arginine
2) glutamate
3) tryptophan

Front 43

what amino acid is a precursor to the following molecule?

tyrosine

Back 43

phenylalanine

Front 44

what is the pathway starting from phenylalanine --> --> --> --> --> Epi

Back 44

phenylalanine --> tyrosine --> dopa --> dopamine --> NE --> epi

Front 45

what amino acid is a precursor to the following molecule?

serotonin (including the cofactor)

Back 45

tryptophan (requires BH4, tetrahydrobiopterin)

Front 46

a pt w/ PKU should have diet low in phenylalanine--> what other dietary modifications should a patient w/ PKU make?

Back 46

tyrosine, BH4 cofactor

Front 47

a 2 y/o girl has an increase in abdominal girth, failure to thrive, and skin/hair depigmentation --> what is her dx?

Back 47

kwashiokor

Front 48

a patient has a genetic dz in which the treatment includes protein restriction to prevent mental retardation, ketoacidosis, and death --> what is the dx?

Back 48

maple syrup urine dz

Front 49

what is the treatment for homocystinuria?

Back 49

decrase methionine, incrase cysteine in diet, B12/folate supplement, and B6 (if deficient)

Front 50

what is the most common urea cycle disorder and how does it present?

Back 50

ornithine transcarbamoylase (OTC) deficiency; interferes w/ body's ability to eliminate ammonia. p/w orotic acid in blood/urine, decreased BUN, symptoms of hyperammonia (tremor, somnolence, vomiting)

Front 51

tx of hyperammonia not 2/2 liver dz?

Back 51

limit protein in diet. benzoate and phenylbutyrate (both of which bind AA and lead to excretion) to decrease ammonia levels.

Front 52

what enzyme allows phenylalanine --> tyrosine?

Back 52

phenylalanine hydroxylase (deficient in PKU)

Front 53

what enzyme allows tyrosine --> dihydroxyphenylalanine ("dopa")

Back 53

tyrosine hydroxylase

Front 54

what enzyme allows dopa --> dopamine?

Back 54

dopa decarboxylase

Front 55

what enzyme allows Norepinephrine---> epinephrine

Back 55

phenylethanol-amine-N-methyl transferase (PNMT) -- this occurs mostly in adrenal medulla.

Front 56

a middle aged man has dark spots on his sclera and has noted that his urine turns black when left sitting for a period of time. dx?

Back 56

alkaptonuria

Front 57

what are the essential fatty acids?

Back 57

linoleic and linolenic acids

Front 58

what do the following apolipoproteins do?

1) A-I
2) B-100
3) C-II
4) B-48
5) E

Back 58

1) Activates LCAT
2) Binds to LDL receptor, mediates VLDL secretion
3) Cofactor for activation of lipoprotein lipase
4) mediates chylomicron secretion
5) mediates Extra (remnant) uptake of VLDL and chylomicrons by liver cells

Front 59

which apolipoprotein:

1) accompanies VLDL from liver to deliver hepatic TGs to peripheral tissue

2) accompanies chylomicrons to deliver dietary TGs from gut to peripheral tissue

Back 59

1) apo B-100
2) apo B-48

Front 60

newborn w/ failure to thrive, fatty stools, intestinal bx shows accumulation of lipids w/i enterocytes --> what is the most likely dx?

Back 60

abeta-lipoproteinemia (hereditary inability to synthesize apoB-100 and apoB-48, can't release VLDL from liver and chylomicrons from intestines)