Liver disease associated with ZZ alpha1-antitrypsin deficiency and ursodeoxycholic acid therapy in children.

doi:10.1097/MPG.0b013e31817b6dfb

Article

Liver disease associated with ZZ alpha1-antitrypsin deficiency and ursodeoxycholic acid therapy in children.

Journal of pediatric gastroenterology and nutrition - 1 Nov 2008

Lykavieris Panayotis, Ducot Béatrice, Lachaux Alain, Dabadie Alain, Broué Pierre, Sarles Jacques, Bernard Olivier, Jacquemin Emmanuel

Abstract excerpt

OBJECTIVES: To investigate the effect of ursodeoxycholic acid (UDCA) in children with liver disease associated with ZZ alpha1-antitrypsin (AAT) deficiency. PATIENTS AND METHODS: A total of 42 affected children received UDCA (30 mg x kg x day(-1)) and underwent clinical and biochemical follow-up at least yearly. RESULTS: In group 1, 22 children whose mean initial gamma-glutamyl-transpeptidase (GGT) was 7.4 x N...

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