PEDIATRICS Vol. 91 No. 5 May 1993, pp. 986-988
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Screening Urine of 3-Week-Old Newborns: Lack of Association Between Sudden Infant Death Syndrome and Some Metabolic Disorders

BERNARD LEMIEUX MD1, ROBERT GIGUERE BSc1, DENIS CYR BSc1, DENIS SHAPCOTT PHD1, MARK MCCANN BSc2, and MENDEL TUCHMAN MD2

1 Quebec Urine Metabolic Screening Program Dept of Pediatrics, University of Sherbrooke Medical Center, Sherbrooke, Quebec, Canada
2 Biochemical Genetics Laboratory, Division of Genetics and Metabolism, Depts of Pediatrics and Laboratory Medicine and Pathology, University of Minnesota, Minneapolis

The only genetic metabolic disorder clearly linked thus far to sudden infant death syndrome (SIDS) is medium-chain acylcoenzyme A dehydrogenase (MCAD) deficiency. There has been no evidence for an association between SIDS and other hereditary metabolic disorders. A few studies, which were often carried out retrospectively on single subjects, have involved the measurement of various metabolites including organic acids, carnitine, free amino acids, and the enzymes implicated in the oxidation of fatty acids, and these have not linked SIDS to inborn errors of metabolism. The study of Harpey et al1 reported that 15% of SIDS infants have a fatty acid beta-oxidation defect.

Submitted on June 4, 1992
Accepted on November 24, 1992