Medium-chain acyl CoA dehydrogenase deficiency: Its relationship to SIDS and the impact on genetic counseling

Sudden infant death syndrome (SIDS) is defined as the sudden and unexpected death of an apparently healthy infant under 1 year of age. Routine autopsies often provide few clues as to the cause of death and rarely include a biochemical evaluation. Genetic counseling for SIDS can be difficult as recurrence risks vary depending on the age at death and the number of deaths which have occurred in the family. Biochemical disorders may account for up to 5% of SIDS. Of the metabolic disorders known to be involved in SIDS, the most commonly found is medium-chain acyl CoA dehydrogenase deficiency (MCAD). MCAD is an autosomal recessive disorder of fatty acid oxidation which accounts for up to 1% of SIDS. For some families, the addition of a postmortem biochemical investigation can identify an unsuspected metabolic disorder as the cause of death. Once the diagnosis is established, accurate genetic counseling can then be provided. Metabolic testing of the surviving siblings of victims of sudden death, and the subsequent identification of those due to MCAD can prevent the tragedy of recurrent SIDS in some families. In addition, screening the survivors of an acute life threatening event (ALTE) may also prevent a recurrence.     

Grading the ‘cultural literacy’ project

The essay examines the argument advanced by E.D. Hirsch, Jr., for instituting ‘cultural literacy’ as a fundamental priority of schools. A number of confusions and equivocations in Hirsch's reasoning are identified, and the propensity of his project to indoctrinate is exposed. Among the features of Hirsch's argument shown to be troubling are his shifting construal of ‘language’, his inconsistency about the requirements of cultural literacy, and his uncritical relation to traditional images of the American past and present. The upshot is to raise the question why Hirsch's project has elicited wide support and praise.