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August 1992

Sudden Infant Death Syndrome in Neonates-Reply

Author Affiliations

University of Florida College of Medicine PO Box 100296 Gainesville, FL 32610
University of Florida College of Medicine Jacksonville Campus 580 W 8th St Jacksonville, FL 32209

Am J Dis Child. 1992;146(8):903-904. doi:10.1001/archpedi.1992.02160200025016

In Reply.Dr Elpeleg correctly states that the metabolic workup for our surviving patients included only screening for urinary reducing substances and an amino acid profile. He states that this is inadequate for diagnosis of defects in mitochondrial β-oxidation of fatty acids, which has been reported to cause SIDS, and suggests that fibroblast culture be performed for all SIDS victims and patients with near-miss cases of SIDS.

The patients we reported were born between October 1985 and April 1990, or before the interesting study by Harpey et al1 was published. Still, it is unlikely that any of our patients had defects in fatty acid β-oxidation because (1) the illness did not progress in survivors, and (2) fatty infiltration of the liver was not present in SIDS victims. Fatty infiltration of the liver, heart, and skeletal muscle is the hallmark of this disorder. Losty et al2 reported an infant with medium-chain

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