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August 1992

Sudden Infant Death Syndrome in Neonates

Author Affiliations

Metabolic Unit Shaare-Zedek Medical Center Jerusalem 91031, Israel

Am J Dis Child. 1992;146(8):903. doi:10.1001/archpedi.1992.02160200025015

Sir.—Burchfield and Rawlings1 reported sudden infant death syndrome (SIDS) or other life-threatening events in 10 newborns during the early neonatal period. Metabolic evaluation was confined to determination of levels of blood gases, urinary reducing substances, and amino acids.

Defects in mitochondrial β-oxidation of fatty acids have recently been recognized as a metabolic cause of SIDS and "near-miss" SIDS. It has been estimated that about 15% of these events may be related to β-oxidation defects.2 Neither the absence of fatty infiltration of the viscera on autopsy3 nor the early onset of the catastrophic events can exclude this group of diagnoses. We encountered two siblings who presented with SIDS at age 2 days less than 3 hours after being fed. In an apparently healthy offspring of the same family, long-chain acyl-CoA dehydrogenase deficiency in fibroblasts was later found.

Because blood glucose levels often remain within the normal range

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