Chronic intrahepatic cholestasis due to sarcoidosis.

Chronic intrahepatic cholestasis due to sarcoidosis.

Two West Indian patients with Kveim-biopsy proven sarcoidosis developed chronic cholestatic liver disease, clinically and biochemically similar to primary biliary cirrhosis. Liver histology revealed multiple granulomas with reduction in bile ducts and, in one patient, progression to biliary cirrhosis. Portal hypertension was present in both patients leading to severe variceal haemorrhage in one...

Exchange transfusion for intrahepatic cholestasis due to sickle beta thalassaemia.

Intrahepatic cholestasis is a rare but potentially fatal complication of sickle cell disease [1]. Sickle cell intrahepatic cholestasis (SCIC) is characterised by right upper quadrant pain, hepatomegaly and progressive hyperbilirubinaemia. To date 15 adult patients have been reported in the literature and only six survived [2]. We present a case of haemoglobin S/Beta thalassaemia with intrahepat...

Progressive Familial Intrahepatic Cholestasis

Intrahepatic cholestasis of pregnancy.

Intrahepatic cholestasis of pregnancy is the most common pregnancy-specific liver disease that typically presents in the third trimester. The clinical features are maternal pruritus in the absence of a rash and deranged liver function tests, including raised serum bile acids. Intrahepatic cholestasis of pregnancy is associated with an increased risk of adverse perinatal outcomes, including spon...

Severe familial intrahepatic cholestasis.

Odievre, M., Gautier, M., Hadchouel, M., and Alagille, D. (1973). Archives of Disease in Childhood, 48, 806. Severe familial intrahepatic cholestasis. Five cases of intrahepatic cholestasis are reported in 4 families in which 7 other children have had the same disease. Cholestasis, beginning before the age of one year, is notable for the absence of lipid retention, evolving by attacks brought o...