LIVER-TRANSPLANTATION FOR NEONATAL HEMOCHROMATOSIS

被引：21

作者：

MUIESAN, P

RELA, M

KANE, P

DAWAN, A

BAKER, A

BALL, C

MOWAT, AP

WILLIAMS, R

HEATON, ND

机构：

[1] UNIV LONDON KINGS COLL HOSP, LIVER TRANSPLANT SURG SERV, LONDON SE5 9RS, ENGLAND

[2] UNIV LONDON KINGS COLL HOSP, DEPT RADIOL, LONDON SE5 9RS, ENGLAND

[3] UNIV LONDON KINGS COLL HOSP, DEPT CHILD HLTH, LONDON SE5 9RS, ENGLAND

[4] UNIV LONDON KINGS COLL HOSP, INST LIVER STUDIES, LONDON SE5 9RS, ENGLAND

来源：

ARCHIVES OF DISEASE IN CHILDHOOD-FETAL AND NEONATAL EDITION | 1995年 / 73卷 / 03期

关键词：

LIVER TRANSPLANTATION; NEONATAL HEMOCHROMATOSIS; FULMINANT HEPATIC FAILURE;

D O I：

10.1136/fn.73.3.F178

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Two cases of neonatal haemochromatosis, a rare and often fatal metabolic disorder, presenting with acute liver failure, are reported. Both presented in the first week of Life with hypoglycaemia, jaundice, and coagulopathy, with rapid deterioration of Liver function. Both received a transplantation using reduced Liver grafts. One child was well 18 months later. Few survivors have been reported and despite the difficult perioperative management, liver transplantation is the best treatment for neonatal haemochromatosis.

引用

页码：F178 / F180

页数：3

共 10 条

[1]

Collins J, 1990, Hepatology, V12, P176, DOI 10.1002/hep.1840120131

[2]

Corbally M T, 1994, Transpl Int, V7 Suppl 1, pS104

[3] FETAL LIVER-DISEASE MAY PRECEDE EXTRAHEPATIC SIDEROSIS IN NEONATAL HEMOCHROMATOSIS [J].