PEDIATRICS Vol. 107 No. 4 April 2001, pp. 809
To the Editor.
The American Academy of Pediatrics (AAP) Task Force on Infant
Sleep Position and Sudden Infant Death Syndrome (SIDS) has identified a
number of risk factors for SIDS, including prone sleeping, SIDS among
siblings, infanticide, and cardiac arrhythmias.1 However,
one important and preventable risk factor was omitted. This factor, a
metabolic disorder of fatty acid oxidation The critical element in prevention is identification through newborn
screening. This is now possible by applying a technology known as
tandem mass spectrometry (MS-MS) to the heel-stick blood specimen
collected for routine newborn screening.5,6 Unfortunately,
relatively few newborn infants are screened in this comprehensive
manner. These include infants born in most (but not all) hospitals in
Pennsylvania and, most recently, those born in Massachusetts. The AAP
would provide a valuable service by advocating the expansion of newborn
screening with MS-MS throughout the United States.
notably, but not
exclusively, medium-chain acyl-CoA dehydrogenase deficiency (MCADD)
is
increasingly recognized as a cause of infant death frequently mistaken
for SIDS2 and as a major cause of sudden death among
siblings.3 In testing 4579 blood specimens obtained from
autopsies of deaths diagnosed as SIDS, Neo Gen Screening, Inc, of
Pittsburgh, Pennsylvania, has identified 21 cases of MCADD as well as
15 cases of other fatty acid oxidation disorders
(www.neogenscreening.com/PostmortemScreeningSummary.htm). Retrospective biochemical screening of postmortem liver specimens equally revealed a high incidence of fatty acid oxidation disorders in
sudden death cases diagnosed as SIDS.4 These deaths from
disorders of fatty acid oxidation are preventable by treatment that
includes avoidance of fasting, carbohydrate supplementation in the
evening, and, perhaps, carnitine.
Children's Hospital
Boston, MA 02115
REFERENCES
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