Problems in Intrahepatic Cholestasis. Second International Symposium on Intrahepatic Cholestasis, Florence 1978

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...

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 ...

Intrahepatic cholestasis in the newborn.

Hitherto little attention has been paid to cholestatic jaundice in the newborn which occurs in the absence of rhesus incompatibility. 4 such cases, among a consecutive series of 24 newborns with prolonged neonatal jaundice occurring in the Devon and Exeter clinical area since 1960, are here described in detail to demonstrate that their clinical and histological picture, and their prognosis, is ...