Oral L‐Alloisoleucine Loading Studies in Healthy Subjects and in Patients with Maple Syrup Urine Disease
作者:
PETER SCHADEWALDT,
CHRISTIAN DALLE-FESTE,
ULRICH LANGENBECK,
UDO WENDEL,
期刊:
Pediatric Research
(OVID Available online 1991)
卷期:
Volume 30,
issue 5
页码: 430-434
ISSN:0031-3998
年代: 1991
出版商: OVID
数据来源: OVID
摘要:
Total body and renal elimination of L-allo-isoleucine was assessed after oral loads (0.57 mmol/kg body wt) in four healthy subjects and in five patients with maple syrup urine disease (MSUD) of different degrees of severity. As judged from the fictive initial concentration, L-alloisoleucine is distributed evenly in the total body water space. In the controls, estimated half-time of total elimination was 9.2 ± 2.2 h (n= 4). In the MSUD patients, it ranged from 26 h (mild variant) to about 8 d (classical type). Because of its low renal clearance rate, L-alloisoleucine was cleared through ketomethylvalerate to >99% in normals and to at least 73% in the MSUD patients. Assuming small variation in the losses of ketomethylvalerate through L-isoleucine formation and through renal excretion, this test allows ranking of MSUD patients with regard to their residualin vivobranched-chain oxo-acid dehydrogenase activity.
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