Liver diseases

Question 1

Sources of ALP

Answer 1

liver, bone, intestine, kidney and placenta

Question 2

Elevated LFTs - causes of hepatocellular injury

Answer 2

Viral (A/B/C/D/E/CMV/EBV/HSV/VZV), AI, drugs/toxins, NAFLD, Vascular, Hereditary (A1AT, Wilson’s, celiac, hemochrom.)

Question 3

Causes of intrahepatic cholestasis

Answer 3

Sex hormones, erthromycin, sepsis, post-op, PBC

Question 4

Cause of elevated liver ALP

Answer 4

HCC, Mets, Lymphoma; TB, sarcoidosis, histoplasmosis;amoebic/bacterial;medications/idiopathic

Question 5

Hepatocellular pattern >2x/ULN

Answer 5

Lifestyle mod, + repeat in 6 mo, screen for CLD causes, if still elevated >2xULN, consider liver bx, otherwise observe.

Question 6

Cholestatic pattern

Answer 6

RUQ U/S, AMA -> if either positive, liver biopsy; if both negative but ALP >1.5x ULN consider Bx, if observe

Question 7

PBC genetic associations

Answer 7

X monosomy, variants in IL12alpha, IL12 receptor genes

Question 8

PBC work up

Answer 8

AMA Ab, IgM, Bili, ALP, cholesterol, liver biopsy if AMA negative, (or ANA+SM AB = AMA)

Question 9

PBC treatment

Answer 9

Ursodeoxycholic acid 15mg/kg/d

Question 10

Benefits of Urso in PBC

Answer 10

Inc. survival, dec. histologic change and complications (varices)

Question 11

Refractory PBC on urso

Answer 11

Colchicine, MTX, budesonide

Question 12

Pruritus treatment in PBC

Answer 12

Cholestyramine give 2-4 hrs after urso, if refractory naltrexone, rifampin

Question 13

Vitamin deficiencies in PBC

Answer 13

Fat soluble vitamins, screen and Rx osteoporosis even if not Vit D deficient

Question 14

PSC work up

Answer 14

MRCP +/- ERCP show multifocal beaded bile duct strictures

Question 15

Liver biopsy features of PSC

Answer 15

Onion skin fibrosis around bile ducts, may exclude diagnosis of autoimmune sclerosing cholangitis

Question 16

PSC treatment

Answer 16

No meds have improved survival. urso may decrease colon CA risk in pts with UC, & improve LFTs in pts w/o UC. Endoscopic dilation in dominant stricture, short term stenting or surgical resection. Colectomy in UC decrease recurrence of PSC post liver transplant

Question 17

CholangioCA surveillance in PSC

Answer 17

Biannual screening with MRCP/ or U/S, CA19-9

Question 18

Indication for HBV treatment

Answer 18

1) if HBeAg positive with DNA >20,000IU/ml and elevated ALT 2) HBeAg negative with DNW >2000IU/ml and elevated ALT or liver biopsy shows stage >/=2 fibrosis

Question 19

Wilson’s disease extrahepatic S/S

Answer 19

Coombs negative hemolytic anemia, renal disease, neuro psych disease, parkinsonism/movement disorder, Kayser-Fleischer rings

Question 20

Wilson’s disease diagnosis

Answer 20

Incr. 24 hr urine copper, decrease serum ceruloplasmin (se 90%), liver biopsy shows copper

Question 21

Wilson’s disease pathophysio

Answer 21

Recessive disorder of copper transport (mutation in ATP7B), affects liver primarily, also brain, eye

Question 22

Wilson’s disease treatment

Answer 22

Chelation with penicillamine + pyridoxine; 2nd line trientine. Zinc can help delay disease best used it asymptomatic or in conjunction with chelation

Question 23

Secondary iron overload

Answer 23

thalassemia, RBC transfusion, MDS, Alcohol, NASH

Question 24

Hemochromatosis treatment

Answer 24

Phlebotomy qwk till Fe Sat

Question 25

Hemochromatosis second line treatment

Answer 25

deferoxamine or deferasirox