B5-026 Bile Duct Disorders Flashcards
- elevated alk phos/GGT
- jaundice/icterus
- pruritus
- skin xanthomas
cholestasis
jaundice occurs when bilirubin exceeds […]
2.5 ish
damaged ducts, hallmark of..
primary biliary cholangitis
classically occurs in middle-aged females
primary biliary cholangitis
aberrant expression of MHC class II on bile duct epithelial cells and autoreactive T cells around ducts
primary biliary cholangitis
MHC class II
M2
E2
all associated with
primary biliary cholangitis
directed against M2 family of mitochondrial antigens, most frequently the E2 part of pyruvate dehydrogenase
AMA
AMA positive
elevated IgM
primary biliary cholangitis
- destructive lymphocytic cholangitis
- florid duct lesions
- granumolas usually
primary biliary cholangitis
florid duct lesions
primary biliary cholangitis
treatment primary biliary cholangitis
ursodeoxycholic acid to slow progression
ursodiol
classically in middle aged men with ulcerative colitis
primary sclerosing cholangitis
- P-ANCA positive
- elevated IgM
primary sclerosing cholangitis
how is primary sclerosing cholangitis linked to UC?
T cells activated in damaged colonic mucosa migrate to the liver and recognize a cross reacting bile duct antigen
associated with HLA-B8
primary sclerosing cholangitis
hallmark is cholangiogram with beaded areas
primary sclerosing cholangitis
progressive concentric periductal firbrosis “onion skin”
primary sclerosing cholangitis
- causes acute bouts of ascending cholangitis
- persistant elevation of alk phos
- increases risk of developing cholangiocarcinoma
primary sclerosing cholangitis
associated with other autoimmune conditions (Hashimoto thyroiditis, rheumatoid arthritis, celiac disease)
primary biliary cholangitis
- abdominal pain radiating to back
- weight loss
- migratory thrombophlebitis
- obstructive jaundice
pacreatic adenocarcinoma
- congenital dilation of common bile duct
- presents in kids under 10 with jaundice and abdominal pain
choledochal cysts
- congenital malformation of the biliary tree
- small bile duct hamartomas
- associated with polycystic liver/kidney disease
- congenital hepatic fibrosis
fibropolycystic disease
congenital hepatic fibrosis
risk factors for cholangiocarcinoma
- primary sclerosis cholangitis
- congenital fibropolycystic disease
- Thorotrast
- clonorchis, opisthorchis liver flukes
cholangiocarcinoma
gene mutation specific to intrahepatic cholangiocarcinoma
loss of BAP1
mutations associated with extrahepatic biliary adenocarcinoma
KRAS
tp53, SMAD4
similar to pancreatic adenocarcinoma
round or multi-faceted
yellow in color
cholesterol stones
caused by increased hepatic cholesterol
risk factors for cholesterol stones
4
- female
- diabetes
- pregnancy
- estrogen
chronic progressive liver disease associated with lymphocytic cholangitis and AMA
PBC
jaundice
pruritis
xanthomas of skin
PBC
p-ANCA is associated with
PSC
concentric periductal fibrosis
PSC
young male with chronically elevated serum alkaline phosphatase
PSC
interface hepatitis
autoimmune hepatitis
chronic Hep C
centrilobular changes with perivenular necrosis
long term acetaminophen use
bland cholestasis
long term anabolic steroid use
majority of patients with PSC have underlying
UC
proliferated bile ductular structures that contain inspissated bile
sepsis
usually caused by gram negative bacteria (E. coli)
panlobular inflammatory infiltrate
acute viral hepatitis
centrilobular necrosis
acetaminophen OD
florid duct lesion with lymphocytis cholangitis
PBC
concentric periductal fibrosis
PSC
bland intrahepatic cholestasis with little inflammation
contraceptives or anabolic steroids
allopurinol is associated with […] in the liver
granulomas
ethanol causes
steatohepatitis
rhodanine stain
wilson’s disease
- fatigue
- abdominal pain
- jaundice
- tremor in hands
- lack of coordination
wilson’s disease
excess copper deposited in liver and brain
Wilson’s
PAS positive, diastase resistant globules in liver
a1-antitrypsin deficiency
characterized by unconjugated hyperbilirubinemia
2
Crigler-Najjar
Gilbert