Liver transplantation for alpha-1-antitrypsin deficiency in children

被引：32

作者：

Prachalias, AA ^{[1
]}

Kalife, M ^{[1
]}

Francavilla, R ^{[1
]}

Muiesan, P ^{[1
]}

Dhawan, A ^{[1
]}

Baker, A ^{[1
]}

Hadzic, D ^{[1
]}

Mieli-Vergani, G ^{[1
]}

Rela, M ^{[1
]}

Heaton, ND ^{[1
]}

机构：

[1] Kings Coll Hosp London, Liver Trnasplant Surg Serv, Paediat Liver Serv, London SE5 9RS, England

来源：

TRANSPLANT INTERNATIONAL | 2000年 / 13卷 / 03期

关键词：

alpha-1-antitrypsin deficiency; liver transplantation; childhood;

D O I：

10.1007/s001470050688

中图分类号：

R61 [外科手术学];

学科分类号：

摘要：

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

引用

页码：207 / 210

页数：4