丙酸血症女童的饮食治疗：补充亮氨酸实现了追赶生长。

Dietary therapy in a girl with propionic acidemia: supplement with leucine resulted in catch up growth.

作者信息

Satoh T, Narisawa K, Tazawa Y, Suzuki H, Hayasaka K, Tada K, Kawakami T

出版信息

Tohoku J Exp Med. 1983 Apr;139(4):411-5. doi: 10.1620/tjem.139.411.

DOI:10.1620/tjem.139.411

Abstract

A 15-month-old girl with propionic acidemia presented a failure to thrive and muscular hypotonia, who had been treated with diets low in leucine, valine, isoleucine, threonine and methionine. Supplementation with leucine gave her a catch up growth and brought about an improvement in muscular hypotonia in parallel with restoration of plasma leucine which had been below normal range during the treatment with the above diets. Oral loading test of leucine produced no ketoacidosis. These findings indicate that the restriction of leucine is not necessary in the dietary management of propionic acidemia.

摘要

一名患有丙酸血症的15个月大女孩出现生长发育迟缓及肌张力减退，此前一直采用低亮氨酸、缬氨酸、异亮氨酸、苏氨酸和蛋氨酸饮食进行治疗。补充亮氨酸后，她实现了追赶生长，并使肌张力减退得到改善，同时血浆亮氨酸水平恢复正常，在上述饮食治疗期间该水平一直低于正常范围。亮氨酸口服负荷试验未引发酮症酸中毒。这些发现表明，在丙酸血症的饮食管理中，无需限制亮氨酸。