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

页码：175 / 179

页数：5

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