THE EFFECTS OF EARLY TREATMENT OF HEREDITARY TYROSINEMIA TYPE-I IN INFANCY BY ORTHOTOPIC LIVER-TRANSPLANTATION

被引：14

作者：

FLYE, MW

RIELY, CA

HAINLINE, BE

SASSA, S

GUSBERG, RJ

BLAKEMORE, KJ

BARWICK, KW

HORWICH, AL

机构：

[1] YALE UNIV,SCH MED,DEPT INTERNAL MED & PEDIAT,NEW HAVEN,CT 06510

[2] YALE UNIV,SCH MED,DEPT PEDIAT,NEW HAVEN,CT 06510

[3] WASHINGTON UNIV,SCH MED,DEPT PEDIAT,ST LOUIS,MO 63110

[4] ROCKEFELLER UNIV,NEW YORK,NY 10021

[5] YALE UNIV,SCH MED,DEPT SURG,NEW HAVEN,CT 06510

[6] YALE UNIV,SCH MED,DEPT HUMAN GENET,NEW HAVEN,CT 06510

[7] YALE UNIV,SCH MED,DEPT PATHOL,NEW HAVEN,CT 06510

来源：

TRANSPLANTATION | 1990年 / 49卷 / 05期

关键词：

D O I：

10.1097/00007890-199005000-00017

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

Two infants with hereditary tyrosinemia secondary to fumarylacetoacetate hydrolase (FAH) deficiency underwent orthotopic liver transplantation at 14 and 16 weeks of age due to poor clinical and biochemical response to medical therapy. Prompt clearance of abnormal metabolites with improved mental alertness and appetite occurred with minimal perioperative complications. Both infants tolerated rapid institution of normal diets and have shown progressive growth and development in the first 36 months after transplantation. Early liver transplantation should be considered as an option for infants with certain inherited metabolic disorders with poor prognosis, such as tyrosinemia type I, who fail to respond to medical therapy. © 1990 by Williams & Wilkins.

引用

页码：916 / 921

页数：6

共 28 条

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