The Turkish Journal of Pediatrics 2005 , Vol 47 , Num 4
Progressive familial intrahepatic cholestasis with normal GGT level appearing with lichenification and enlargement of hands and feet
1Department of Pediatric Gastroenterology, Social Security Children’s Hospital, Gazi University Faculty of Medicine, Ankara, Turkey
2Department of Pathology, Gazi University Faculty of Medicine, Ankara, Turkey
Doğancı T, Akyol G, Bulaç S. Progressive familial intrahepatic cholestasis with normal GGT level appearing with lichenification and enlargement of hands and feet. Turk J Pediatr 2005; 47: 385-389.

Progressive familial intrahepatic cholestasis is a serious disease of the liver, known as Byler disease, characterized by hepatocellular cholestasis. Severe pruritus and high serum bile acid concentrations are the most important diagnostic criteria of this autosomal recessive inherited disease. Here, we present a five-year-old boy with lichenification and enlargement of hands and feet as a sign of progressive familial intrahepatic cholestasis due to severe pruritus. Keywords : progressive familial intrahepatic cholestasis, GGT, enlargement of extremities

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