biliary disorders Flashcards
what are the key findings clinical associated with biliary atresia ?
jaundice
dark urine
pale stools (acholic stools)
what are the ultrasound findings associated with biliary atresia ?
absent gall bladder or abnormal
what is the surgery for biliary atresia ?
kasai procedure
when does biliary atresia present ?
after 2 weeks of life
what is primary biliary cirrhosis ?
autoimmune disorder
t cells attack on small intralobular bile ducts
causes granulomatous inflammation
biliary cirrhosis without extra hepatic obstruction
what are the clinical features associated with PBC ?
more common among women
fatigue and pruritus
itching first then jaundice
itching may be severe at night
how is a diagnosis of PBC made ?
anti mitochondrial antibodies
marked elevated ALP
serum lipids are increased (xanthoma)
what imaging findings are associated PBC ?
imaging shows absence of biliary obstruction
liver biopsy is gold standard
what is the treatment for PBC ?
UDCA - urosidol
liver transplantation
what antibody is elevated with PBC ?
IgM
what other diseases are associated with PBC ?
other autoimmune disorders
most commonly sjogrens
what is primary sclerosing cholangitis ?
autoimmune disorder
inflammation of the biliary tree
involves the intra and extra hepatic bile ducts
what disease is highly associated with primary sclerosing cholangitis ?
strongly associated with ulcerative colitis
patient with ulcerative colitis w esfar ?
primary sclerosing cholangitis
what are the lab findings associated with primary sclerosing cholangitis ?
cholestatic pattern - raised ALP , raised GGT , raised conjugated bilirubin
elevated IgM
positive p-ANCA
what are the biopsy findings associated with primary sclerosing cholangitis ?
onion skin appearance - due to periductal fibrosis
what imaging confirms PSC ?
cholangiogram
shows a beading pattern ( beads on a string appearance)
what is the treatment for PSC ?
endoscopic therapy
liver transplantation
what must be screened for in primary sclerosing cholangitis ?
cholangiocarcinoma
what is the difference between cholangiocarcinoma and gall bladder carcinoma ?
cholangiocarcinoma is a cancer of the epithelial duct cells
gall bladder cancer - arises from the gall bladder itself
what are the key risk factors associated with cholangiocarcinoma ?
chronic biliary inflammation
PSC - in association with UC
clonorchis sinensis ( chinese liver fluke)
