BRANCHED-CHAIN AMINO-ACID FREE PARENTERAL-NUTRITION IN THE TREATMENT OF ACUTE METABOLIC DECOMPENSATION IN PATIENTS WITH MAPLE SYRUP URINE DISEASE

被引：26
|
作者：
BERRY, GT [1 ]
HEIDENREICH, R [1 ]
KAPLAN, P [1 ]
LEVINE, F [1 ]
MAZUR, A [1 ]
PALMIERI, MJ [1 ]
YUDKOFF, M [1 ]
SEGAL, S [1 ]
机构：
[1] UNIV PENN,SCH MED,DEPT PEDIAT & MED,PHILADELPHIA,PA 19104
来源：
NEW ENGLAND JOURNAL OF MEDICINE | 1991年 / 324卷 / 03期
关键词：
D O I：
10.1056/NEJM199101173240307
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
MAPLE syrup urine disease is a rare inborn error of branched-chain amino acid and branched-chain keto acid metabolism due to decreased branched-chain α-keto acid dehydrogenase-complex activity.1 The branched-chain keto acids, which are the substrates in this irreversible enzyme reaction, are derived from transamination of the branched-chain amino acids leucine, isoleucine, and valine. Consumption of branched-chain amino acids in the form of dietary protein in excess of an age-dependent daily requirement for protein synthesis results in the accumulation of branched-chain amino acids and branched-chain keto acids in the tissues and body fluids of patients in whom there is little or no. . . © 1991, Massachusetts Medical Society. All rights reserved.
引用
收藏
页码：175 / 179
页数：5
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