Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare hepatic disease characterized by intrahepatic cholestasis and deterioration of liver function in patients receiving parenteral nutrition for extended periods of time (signs may appear as early as within the first two weeks of initiation of parenteral nutrition). The condition commonly occurs in neonates and usually resolves with transition to enteral feeding, although severe cases may progress to liver fibrosis, cirrhosis, and portal hypertension. Disease data Classification Particular clinical situation in a disease or syndrome Synonyms PNAC PNAC ORPHA code 567983 OMIM code - ICD10 code K76.8 ICD11 code DB99.60 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl