Liver transplantation for alpha-1-antitrypsin deficiency in children.

摘要: Alpha-1-antitrypsin (a1-AT) deficiency is an inborn error of metabolism, which can cause liver disease. The condition one the most common genetic disorders in Caucasian population. Here we review our experience with 21 children suffering from end-stage disease due to a1-AT deficiency. All are PIZZ homozygotes. Nineteen them initially presented neonatal jaundice and two hepatosplenomegaly childhood. Twenty-five transplantions were performed. currently alive at a median follow-up 40 months. Liver replacement provides only definite treatment for associated Excellent results be achieved by reducing waiting time transplantation early referral transplant centre.