Mksap 08-31

Front 1

What gene is deficient in hemochromatosis?

Back 1

HFE

Front 2

When can hemochromatosis be treated, and how?

Back 2

Before cirrhosis: with phlebotomy (after cirrhosis, phlebotomy doesn’t decrease risk of HCC)

Front 3

What does hemochromatosis cause? (5)

Back 3

Bronze skin, diabetes, cirrhosis, arthritis, heart failure

Front 4

What is the diagnostic test for hemochromatosis?

Back 4

Liver biopsy

Front 5

How to test for hep A?

Back 5

Look for IgM against Hep A

Front 6

What is the sensitivity of Hep C Ab?

Back 6

Very poor (40% have negative results) ==> need to test for HCV RNA

Front 7

How to test for hep C?

Back 7

Hep C viral load -- look for HCV RNA (not Ab test b/c of poor sensitivity)

Front 8

Classic demographics for PBC

Back 8

middle-aged female

Front 9

Screening test for PBC?

Back 9

AMA (anti-mitochondrial antibody)

Front 10

What does IgG to HAV suggest?

Back 10

old infection / immunity

Front 11

when does HELLP syndrome occur?

Back 11

3rd trimester of pregnancy

Front 12

TTP vs HUS

Back 12

both have MAHA and thrombocytopenia; HUS has renal failure; TTP is +/- fever, MS changes, and renal failure

Front 13

definition of fulminant hepatic failure

Back 13

encephalopathy within 8 wks of liver injury

Front 14

treatment of fulminant hepatic failure

Back 14

transplant b/c of very high mortality rate

Front 15

alcoholic hep vs acetaminophen tox (2)

Back 15

EtOH: moderately elevated AST/ALT (<500), FEVER

Front 16

what is gilbert's syndrome?

Back 16

congenital unconjugated hyperbilirubinemia found in 5% of the population

Front 17

lab findings in PBC

Back 17

cholestatic picture -- elevated AlkPhos and maybe bili

Front 18

how do you calculate globulin level?

Back 18

total serum protein minus albumin

Front 19

what liver dz a/w elevated globulins?

Back 19

AIH

Front 20

mgmt of NASH

Back 20

wt loss

Front 21

primary w/u of labs showing cholestatic picture?

Back 21

RUQ u/s

Front 22

secondary w/u of labs showing cholestatic picture?

Back 22

depends on RUQ u/s findings: if biliary dilitation, ERCP/MRCP; if evidence of infiltrative liver dz, liver biopsy

Front 23

Recommended kCal deficit for weight loss

Back 23

-500 to -1000 kCal/day ==> 1-2lb loss/wk

Front 24

what drugs for wt loss? When to use them?

Back 24

orlistat and silbutramine: only used in pts refractory to diet/exercise, and only serve as temporary measure (wt comes back once drugs d/c'ed)

Front 25

aphthous ulcers are a/w what GI pathology?

Back 25

IBD

Front 26

what is a major non-neoplastic cause of hyperprolcatinemia?

Back 26

hypothyroidism (TRH stims both TSH and prolactin release)

Front 27

lab findings in primary vs secondary hyperprolactinema

Back 27

secondary has lower levels of prolactin production (<200ng/ml)

Front 28

test to dx cushing's dz

Back 28

24hr urinary free cortisol

Front 29

risk factors for esophageal carcinoma

Back 29

white male, long-standing GERD, barrett's esophagus

Front 30

what condition generaly necessary for infectious esophagitis

Back 30

immunocompromise (eg CMV esophagitis in AIDS)

Front 31

MCC relapse of sx in mgmt of Celiac dz?

Back 31

dietary incompliance with gluten-free diet

Front 32

anti-tissue trasnglutaminase used to dx which dz?

Back 32

celiac dz

Front 33

villous blunting a/w? (3)

Back 33

1) CELIAC SPRUE 2) TROPICAL SPRUE 3) BACTERIAL OVERGROWTH

Front 34

tx of tropical sprue

Back 34

folic acid + tetracycline

Front 35

tests for bacterial overgrowth (2)

Back 35

glucose hydrogen breath test, OR lactulose hydrogen breath test

Front 36

IBS not a/w what common GI sx?

Back 36

weight loss

Front 37

usual location of primary anal fissure

Back 37

posterior, midline; anything else suggestive of 2ndary cause, esp Crohn's (66% posterior midline, but 33% not)

Front 38

Mechanism of hydroxyurea

Back 38

ribonucleotide reductase inhibitor --> increased HbF

Front 39

risks of hydroxyurea

Back 39

mild! Can cause reversible myelosuppression, increased risk of AML in pts with polycythemia vera

Front 40

lethal late complication seen in hodgkin's dz survivors treated with multimodal tx (pancytopenia): what is it and when does it occur?

Back 40

secondary myelodysplastic syndrome, usually 3-11 yrs after tx

Front 41

viral cause of anemia in immunocompromised pts?

Back 41

parovirus b19 (reticulpenia)

Front 42

dx of parvovirus anemia

Back 42

parvovirus DNA in blood/marrow, detected via PCR

Front 43

tx of parvovirus anemia

Back 43

IV Ig containing anti-parvoviral IgG

Front 44

Tx of choice for aplastic anemia? Which pts? Survival?

Back 44

allogenic SCT; pts < 50yo with HLA-matched siblings; survival 75-90%

Front 45

tx of chemo-induced anemia

Back 45

weekly EPO

Front 46

what does increased ferritin and decreased Fe levels in anemic pts with chronic inflam dz (eg RA) suggest?

Back 46

Fe deficiency OR ACD: Although labs c/w ACD, could also be Fe deficiency since ferritin is an acute phase reactant

Front 47

what does elevated HbA2 (alpha2delta2) suggest?

Back 47

B-thal (trait)

Front 48

lab findings in alpha-thal vs beta-thal

Back 48

beta-thal: elevated HbA2 (alpha2delta2)

Front 49

epi of myelodysplastic syndromes

Back 49

>50yo

Front 50

MCV in myelodysplastic syndromes

Back 50

increased

Front 51

increased total protein and decreased albumin levels suggests what with bone pathology?

Back 51

elevated globulins -- multiple myeloma

Front 52

hypercalcemia + anemia --> ?

Back 52

?multiple myeloma (look for renal dysfunction)

Front 53

nucleated RBCs seen when?

Back 53

1) Hematologic causes (sickle cell, thal); 2) Marrow infiltrates (myelofibrosis, mets to bone)