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This Concept Map, created with IHMC CmapTools, has information related to: pathology of biliary tract diseases, bile duct obstruction course bile duct proliferation, choleangitis - infxn of biliary tree (PMN's) usu always found in liver biopsies, not diagnostic of bile duct obstruction, cholestasis usu always found in liver biopsies, not diagnostic of bile duct obstruction, primary biliary cirrhosis lymphocytes diagnostic test is AMA-M2 antimitochrondrial autoantibody, M2 autoantigen. 95-98% specific, primary sclerosing cholangitis prognosis 12yr median survival biliary cirrhosis: 3-5yrs transplantation cholangiocarcinoma, primary sclerosing cholangitis clinical presentation symptomatic pruritus jaundice hepatomegaly splenomegaly recurrent cholangitis complications of chronic liver disease, bile ducts disappear -> biliary cirrhosis w/ PHTN looks same as alcoholic cirrhosis, bile duct involvement intra-hepatic bile duct primary biliary cirrhosis lymphocytes, primary sclerosing cholangitis course bile ducts disappear -> biliary cirrhosis w/ PHTN, primary biliary cirrhosis lymphocytes course, progressesion bile ducts disappear -> biliary cirrhosis w/ PHTN, primary sclerosing cholangitis clinical presentation young men asymp abn liver test -esp alk phos, bile lakes - popped bile duct diagnostic of bile duct obstruction, primary biliary cirrhosis lymphocytes tx immunosuppressive agents- corticosteroids, azathioprine, cyclosporin antifibrogenic agents (colchicine) bile acids cholestyramine ursodeoxycholic acid, primary biliary cirrhosis lymphocytes presentation elevated (or nml) alkaline phosphatase AST & ALT 2-3x's nml selective IgM elevation (95%) hypercholesterolemia: el HDL albumin, prothrombin time normal initially, primary biliary cirrhosis lymphocytes pathophysiology autoimmune: lymphocytes target duct epi - seen in GVHD, trnsplnt rejctn antigen to mitochondria cirrhosis only in adv stage of disease, choleangitis - infxn of biliary tree (PMN's) non-suppurative, chronic, destructive primary biliary cirrhosis lymphocytes, bile duct involvement extra-hepatic bile duct primary sclerosing cholangitis, primary sclerosing cholangitis tx rx: cholestyramine immunosuppressive agents surgical (biliary tract reconstruction, transplant), primary sclerosing cholangitis labs alk phos ɯx nml slight abn AST, ALT elevated bilirubin 67% - PBC, el only in adv PBC pANCA perinuclr antineutrophilic cytoplasmic antibodies, primary biliary cirrhosis lymphocytes occurs in women 30-70 9x's more than men