Progressive neurologic disability in methylmalonic acidemia despite transplantation of the liver

被引：82

作者：

Nyhan, WL

Gargus, JJ

Boyle, K

Selby, R

Koch, R

机构：

[1] UCSD Pediat 0830, La Jolla, CA 92093 USA

[2] Univ Calif Irvine, Dept Pediat, Irvine, CA 92717 USA

[3] Univ So Calif, Sch Med, Dept Pediat, Los Angeles, CA 90033 USA

来源：

EUROPEAN JOURNAL OF PEDIATRICS | 2002年 / 161卷 / 07期

关键词：

methylmalonic acidemia; neurologic degeneration; renal failure; transplantation of liver;

D O I：

10.1007/s00431-002-0970-4

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Methylmalonic acidemia unresponsive to cobalamin is often fatal in infancy. Patients have been considered candidates for hepatic transplantation and experience has been that the procedure eliminates the life-threatening episodes of ketoacidosis that characterize this disease. Conclusion: experience with a 24-year-old patient treated with hepatic transplantation indicates that this procedure does not prevent progressive renal failure and neurologic dysfunction.

引用

收藏

页码：377 / 379

页数：3

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