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
