title: [Cholestatic liver disease in children].
abstract: Cholestatic liver disease constitutes a large part of chronic liver diseases during infancy. It is caused either by extrahepatic disorders (obstruction) or by intrahepatic cholestasis (functional). The differential diagnosis should be done as early as possible because the delayed surgical therapy in extrahepatic cholestasis has a very bad prognosis. Intrahepatic cholestasis may be caused by a broad spectrum of different disorders such as congenital infection, endocrine, chromosomal abnormalities or inborn errors of metabolism. Familial clustering is typical for the Byler's disease, Alagille's syndrome, PFIC, errors of bile acid synthesis or alpha-1-ATD. The established diagnosis allows to start etiological treatment: dietary--in metabolic diseases, antibiotics--in bacterial infections, antiviral--in viral infections etc. To lower bile acids level pharmacological treatment (UDCA) or surgical procedures (hepatoportoenterostomy, partial biliary diversion) should be performed. In progressive cholestasis the only effective therapy is liver transplantation.
authors.affiliation: Kliniki Gastroenterologii, Hepatologii i Zywienia, Instytutu - Pomnika Centrum Zdrowia Dziecka w Warszawie.
authors.forename: Irena
authors.initials: I
authors.lastname: Jankowska
authors.forename: Joanna
authors.initials: J
authors.lastname: Pawłowska
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Swiatkowska
authors.forename: Jolanta
authors.initials: J
authors.lastname: Rujner
authors.forename: Jerzy
authors.initials: J
authors.lastname: Socha
mesh_terms: D000367:Age Factors; D001662:Biliary Tract Surgical Procedures; D002648:Child; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D001651:Cholestasis, Extrahepatic / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D002780:Cholestasis, Intrahepatic / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D003937:Diagnosis, Differential; D006801:Humans; D008099:Liver / Q000378:metabolism / Q000473:pathology; D012307:Risk Factors; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid