Although unexpected infant death in a cot has traditionally been attributed to sudden infant death syndrome, careful evaluation of death scenes and sleeping environments has increasingly identified deaths due to accidental asphyxia from so-called sleeping accidents. The case of a 5-month-old infant boy who was found facedown and unresponsive in a wooden portable cot with a sagging canvas base is reported to illustrate another potentially lethal situation. Although the autopsy revealed no specific findings, examination of the cot showed a significant depression caused by the sagging canvas base that was exacerbated by a soft-foam mattress and layers of bedding. Once in the trough, the infant would not have been able to extricate himself. Death was therefore attributed to accidental suffocation due to the infant's position resulting in contact of the mouth and face with soft bedding. In addition to again demonstrating the potential dangers of using old second-hand cots, this case clearly shows the problems that may exist when soft and sagging bedding forms a central trough that may entrap an infant. Death scene investigators should specifically comment on the presence of such troughs and measure of depth of the trough and/or cot base to provide some quantification of the degree of concavity present.
Pseudoxanthoma elasticum (PXE) is a generalized connective tissue disorder in which there is calcification of elastic fibers within arteries, eyes, and skin. Characteristic features include yellow-orange papular skin lesions, angioid streaks radiating out from the optic discs, and arterial calcification. The prevalence in the general population varies widely from 1/70,000 to 1/160,000. PXE has an autosomal recessive inheritance pattern and results from mutations in the ATP-binding cassette transporter C6 (ABCC6) that has been mapped to 16p13.1. Over 300 loss-of-function mutations have been identified. Individuals with PXE may come to forensic attention because of sudden death involving accelerated coronary atherosclerosis with acute myocardial ischemia, systemic hypertension, mitral valve prolapse, restrictive cardiomyopathy, gastrointestinal hemorrhage, and cerebral ischemia or hemorrhage. Because of the heritable nature of the disease, family counseling and screening are in order when previously unsuspected cases are encountered at autopsy.
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