Biliary Tree Conditions Flashcards
(42 cards)
What is primary biliary sclerosis
Immune system attacks the small bile ducts in the liver
Presentation of primary biliary sclerosis
Fatigue, pruritis, GI disturbance and abdo pain, jaundice, pale stools, xanthoma, xanthelasma, signs of cirrhosis and failure
Associations with primary biliary sclerosis
Middle aged women, other autoimmune diseases, rheumatoid conditions
Diagnosis of primary biliary sclerosis
Liver function tests, autoantibodies, ESR raised and IGM raised, liver biopsy
What will be found in the LFTs of primary biliary sclerosis
Alkaline phosphatase will be raised, other liver enzymes and bilirubin are raised in later disease.
What autoantibodies are associated with primary biliary sclerosis
Anti-mitochonrial antibodies (most specific), anti-nuclear antibodies (35% patients)
Treatment of primary biliary sclerosis
Ursodeoxycholic acid, colestyramine, immunosupression in some patients and liver transplant
What does ursodeoxycholic acid do
reduces the intestinal absorption of cholesterol
what does colestyramine do
bile acid sequestrate in that it binds to bile acids to prevent absorption in the gut and can help with pruritis due to raised bile acids
End results of primary biliary sclerosis
Advanced liver cirrhosis and portal hypertension
Complications of primary biliary sclerosis
symptomatic pruritis, fatigue, steatorrhoea, distal renal tubular acidosis, hypothyroidism, osteoporosis, hepatocellular carcinoma
What is primary sclerosing cholangitis
Condition where the intrahepatic or extrahepatic ducts become strictured and fibrotic
What is the pathogenesis behind primary sclerosing cholangitis
There is obstruction to the flow of the bile out of the liver and into the intestines. This leads to liver inflammation, fibrosis and cirrhosis.
Risk factors of primary sclerosing cholangitis
Male, aged 30-40, ulcerative cholitis, family history
Presentation of primary sclerosing cholangitis
jaundice, chronic right upper quadrant pain, pruritis, fatigue, hepatomegaly
What do liver function tests show in primary sclerosing cholangitis
Alkaline phosphatase is most derranged LFT and may be the only abnormality at first. May be a rise in bilirubin, and ALT and AST can be derranged as disease progresses.
What role do autoantibodies have in primary sclerosing cholangitis
None are highly sensitive but some may indicate whether they may respond to immunosuppression
What is the gold standard investigation for primary sclerosing cholangitis
MRCP (magnetic resonance cholangiopancreatography)
Associations and complications of primary sclerosing cholangitis
Acute bacterial cholangitis, cholangiocarcinoma, colorectal cancer, cirrhosis and liver failure, biliary strictures, fat soluble vit deficiencies
Management of PSC
Liver transplant, ERCP, colestyramine, monitoring for complications
What does ERCP do
Dilate and stent any strictures
What is ascending cholangitis
Bacterial infection of the biliary tree
Triad of symptoms in ascending cholangitis
Charcot’s triad - RUQ pain, jaundice, fever
Symptoms in ascending cholangitis
Charcot’s triad, hypotension, tachycardia, confusion
