Increased Urinary Metabolite Excretion during Fasting in Disorders of Propionate Metabolism
作者:
G N THOMPSON,
R A CHALMERS,
期刊:
Pediatric Research
(OVID Available online 1990)
卷期:
Volume 27,
issue 4
页码: 413-416
ISSN:0031-3998
年代: 1990
出版商: OVID
数据来源: OVID
摘要:
Odd-chain fatty acids are recognized precursors of propionate in man, but their clinical significance in disorders of propionate metabolism has not been well studied. Urinary excretion of methylmalonate, methylcitrate, propionylglycine, and 3-hydroxypropionate was measured in five children with methylmalonic acidemia and three with propionic acidemia during frequent or continuous feeding and after 10-18 h of fasting. There was a significant (p<0.01) increase in the mean total measured metabolite excretion during fasting (fed 38.1 µmol/kg/h, fasting 54.6 in methylmalonic acidemia, fed 1.45, fasting 2.98 in propionic acidemia). Percentage rises in each subject were similar for all measured metabolites. These increases in metabolite excretion are most easily explained by mobilization and oxidation of odd-chain fatty acids in the fasting state. Prolonged fasting should be avoided in children with disorders of propionate metabolism.
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