Living-related liver transplantation for neonatal-onset propionic acidemia

被引：33

作者：

Yorifuji, T

Muroi, J

Uematsu, A

Nakahata, T

Egawa, H

Tanaka, K

机构：

[1] Kyoto Univ Hosp, Dept Pediat, Kyoto 6068507, Japan

[2] Kyoto Univ Hosp, Dept Transplantat Immunol, Kyoto 6068507, Japan

来源：

JOURNAL OF PEDIATRICS | 2000年 / 137卷 / 04期

关键词：

D O I：

10.1067/mpd.2000.108391

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

We report a child with neonatal-onset propionic acidemia treated with living-related liver transplantation. Despite minimal improvement in the levels of circulating propionyl CoA metabolites, hyperammonemia was corrected, and no episode of metabolic decompensation was experienced after the transplantation was performed. Natural protein intake could be increased from 0.5 g/kg per day to 2 g/kg per day Anemia was corrected, and the growth rate and mental development improved significantly.

引用

页码：572 / 574

页数：3

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