Sudden Infant Death Syndrome in Neonates

Sir. —Burchfield and Rawlings 1 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 autopsy 3 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