Familial Benign Recurrent Intrahepatic Cholestasis

Progressive Familial Intrahepatic Cholestasis

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...

Progressive familial intrahepatic cholestasis

Progressive familial intrahepatic cholestasis (PFIC) refers to a heterogeneous group of autosomal-recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. The exact prevalence remains unknown, but the estimated incidence varies between 1/50,000 and 1/100,000 births. Three types of PFIC have been identified and associated with mutations ...

"an iranian girl with benign recurrent intrahepatic cholestasis "

this report presents an 11 year-old girl with benign recurrent cholestasis (bric) who developed episodes of severe jaundice and pruritus at the ages of 2.5 and 10 years. each episode lasted for 3-4 months. the peak level of serum bilirubin reached 33-37 mg/dl.liver function tests were all normal during the attack except for increased alkaline phosphatase and prolonged prothrombin time responsiv...

Familial Intrahepatic Cholestasis: An Update

Familial intrahepatic cholestasis is a confusing group of syndromes. Four forms are defined and discussed in detail ("arteriohepatic dysplasia," the Byler syndrome, the THCA syndrome, and Norwegian cholestasis). A comparison of the distinguishing characteristics of these syndromes demonstrates that they share many features, including areflexia, retinal degeneration, and paucity of the intrahepa...