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GIR Test 3 > B5-026 Bile Duct Disorders > Flashcards

B5-026 Bile Duct Disorders Flashcards

1
Q
  • elevated alk phos/GGT
  • jaundice/icterus
  • pruritus
  • skin xanthomas
A

cholestasis

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2
Q

jaundice occurs when bilirubin exceeds […]

A

2.5 ish

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3
Q

damaged ducts, hallmark of..

A

primary biliary cholangitis

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4
Q

classically occurs in middle-aged females

A

primary biliary cholangitis

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5
Q

aberrant expression of MHC class II on bile duct epithelial cells and autoreactive T cells around ducts

A

primary biliary cholangitis

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6
Q

MHC class II
M2
E2

all associated with

A

primary biliary cholangitis

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7
Q

directed against M2 family of mitochondrial antigens, most frequently the E2 part of pyruvate dehydrogenase

A

AMA

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8
Q

AMA positive
elevated IgM

A

primary biliary cholangitis

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9
Q
  • destructive lymphocytic cholangitis
  • florid duct lesions
  • granumolas usually
A

primary biliary cholangitis

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10
Q

florid duct lesions

A

primary biliary cholangitis

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11
Q

treatment primary biliary cholangitis

A

ursodeoxycholic acid to slow progression

ursodiol

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12
Q

classically in middle aged men with ulcerative colitis

A

primary sclerosing cholangitis

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13
Q
  • P-ANCA positive
  • elevated IgM
A

primary sclerosing cholangitis

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14
Q

how is primary sclerosing cholangitis linked to UC?

A

T cells activated in damaged colonic mucosa migrate to the liver and recognize a cross reacting bile duct antigen

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15
Q

associated with HLA-B8

A

primary sclerosing cholangitis

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16
Q

hallmark is cholangiogram with beaded areas

A

primary sclerosing cholangitis

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17
Q

progressive concentric periductal firbrosis “onion skin”

A

primary sclerosing cholangitis

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18
Q
  • causes acute bouts of ascending cholangitis
  • persistant elevation of alk phos
  • increases risk of developing cholangiocarcinoma
A

primary sclerosing cholangitis

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19
Q

associated with other autoimmune conditions (Hashimoto thyroiditis, rheumatoid arthritis, celiac disease)

A

primary biliary cholangitis

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20
Q
  • abdominal pain radiating to back
  • weight loss
  • migratory thrombophlebitis
  • obstructive jaundice
A

pacreatic adenocarcinoma

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21
Q
  • congenital dilation of common bile duct
  • presents in kids under 10 with jaundice and abdominal pain
A

choledochal cysts

22
Q
  • congenital malformation of the biliary tree
  • small bile duct hamartomas
  • associated with polycystic liver/kidney disease
  • congenital hepatic fibrosis
A

fibropolycystic disease

23
Q
A

congenital hepatic fibrosis

24
Q

risk factors for cholangiocarcinoma

A
  • primary sclerosis cholangitis
  • congenital fibropolycystic disease
  • Thorotrast
  • clonorchis, opisthorchis liver flukes
25
Q
A

cholangiocarcinoma

26
Q

gene mutation specific to intrahepatic cholangiocarcinoma

A

loss of BAP1

27
Q

mutations associated with extrahepatic biliary adenocarcinoma

A

KRAS
tp53, SMAD4

similar to pancreatic adenocarcinoma

28
Q

round or multi-faceted
yellow in color

A

cholesterol stones

caused by increased hepatic cholesterol

29
Q

risk factors for cholesterol stones

4

A
  • female
  • diabetes
  • pregnancy
  • estrogen
30
Q

chronic progressive liver disease associated with lymphocytic cholangitis and AMA

A

PBC

31
Q

jaundice
pruritis
xanthomas of skin

A

PBC

32
Q

p-ANCA is associated with

A

PSC

33
Q

concentric periductal fibrosis

A

PSC

34
Q

young male with chronically elevated serum alkaline phosphatase

A

PSC

35
Q

interface hepatitis

A

autoimmune hepatitis
chronic Hep C

36
Q

centrilobular changes with perivenular necrosis

A

long term acetaminophen use

37
Q

bland cholestasis

A

long term anabolic steroid use

38
Q

majority of patients with PSC have underlying

A

UC

39
Q

proliferated bile ductular structures that contain inspissated bile

A

sepsis

usually caused by gram negative bacteria (E. coli)

40
Q

panlobular inflammatory infiltrate

A

acute viral hepatitis

41
Q

centrilobular necrosis

A

acetaminophen OD

42
Q

florid duct lesion with lymphocytis cholangitis

A

PBC

43
Q

concentric periductal fibrosis

A

PSC

44
Q

bland intrahepatic cholestasis with little inflammation

A

contraceptives or anabolic steroids

45
Q

allopurinol is associated with […] in the liver

A

granulomas

46
Q

ethanol causes

A

steatohepatitis

47
Q

rhodanine stain

A

wilson’s disease

48
Q
  • fatigue
  • abdominal pain
  • jaundice
  • tremor in hands
  • lack of coordination
A

wilson’s disease

49
Q

excess copper deposited in liver and brain

A

Wilson’s

50
Q

PAS positive, diastase resistant globules in liver

A

a1-antitrypsin deficiency

51
Q

characterized by unconjugated hyperbilirubinemia

2

A

Crigler-Najjar
Gilbert

GIR Test 3 (16 decks)

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