Cholestasis in children with portal vein obstruction

We describe cholestasis as a result of bile duct abnormalities in 8 children with portal vein obstruction. In a clinical, biochemical and radiological investigation of 121 children with cavernous transformation of the portal vein seen between 1986 and 2000, 8 presented with jaundice, pruritus, and/o...

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Bibliographic Details
Published in:The Journal of pediatrics 2005-04, Vol.146 (4), p.568-573
Main Authors: Gauthier-Villars, Marion, Franchi, Stéphanie, Gauthier, Frédéric, Fabre, Monique, Pariente, Danièle, Bernard, Olivier
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child
Child, Preschool
Cholestasis - etiology
Female
Gastroenterology. Liver. Pancreas. Abdomen
General aspects
Humans
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Male
Medical sciences
Other diseases. Semiology
Portal Vein - surgery
Vascular Diseases - complications
Vascular Diseases - surgery
Vascular Surgical Procedures
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Summary:We describe cholestasis as a result of bile duct abnormalities in 8 children with portal vein obstruction. In a clinical, biochemical and radiological investigation of 121 children with cavernous transformation of the portal vein seen between 1986 and 2000, 8 presented with jaundice, pruritus, and/or raised serum aminotransferases and/or gamma glutamyl transpeptidase (gamma GT) activities. Each displayed dilation and narrowing of intra- and/or extrahepatic bile ducts. Surgical decompression of the portal system (portal-systemic or Rex anastomosis) resulted in the regression of the signs of cholestasis in all children. We conclude that children with portal vein obstruction may exhibit clinically significant cholestasis as a result of external compression of the bile duct by the cavernoma.
ISSN:0022-3476
1097-6833
DOI:10.1016/j.jpeds.2004.12.025