M. Levene
May 28, 1977
Citations
0
Influential Citations
27
Citations
Quality indicators
Journal
The Lancet
Abstract
There are few similarities with the acids reported in Reye’s syndrome by Tanaka et al.12 or by Harrington et al.14 The unusual occurrence, in the urine of both children at admission, of a high concentration of an acid provisionally identified as 4-hydroxy-3-methylpentanoic acid may indicate the underlying cause of their illness. The immediate metabolic precursor of this acid could be 3-methyl-pent-3-enoic acid which could itself be derived from 2-amino-4-methyl-hex-4-enoic acid, an analogue of hypoglycin. This unsaturated aminoacid and other hypoglycin analogues have been reported in a wide variety of plants including the seeds and fruit of the Aceraceæ (maple, sycamore) and Aesculus (horse chestnut) families.15 These could be easily ingested by small infants and children, although there is no evidence that these twins did this. These compounds, like hypoglycin, exert their toxic effects via the coenzyme A and carnitine esters of the unsaturated organicacid metabolites, inhibiting both fatty acid &bgr;-oxidation and gluconeogenesis and causing the abnormal dicarboxylicaciduria, the profound hypoglycaemia, and the other symptoms common to both J.v.s. and Reye’s syndrome in infants. 12