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Death Due to Microvascular Occlusion in Sickle-Cell Trait Following Physical Exertion

NCJ Number
187631
Journal
Journal of Forensic Sciences Volume: 46 Issue: 2 Dated: March 2001 Pages: 399-401
Author(s)
Darren P. Wirthwein M.D.; Sheila D. Spotswood M.D.; Jeffrey J. Barnard M.D.; Joseph A. Prahlow M.D.
Date Published
March 2001
Length
3 pages
Annotation
This paper reports the author's experience with exercise-related deaths in individuals with sickle-cell trait as seen in a medical examiner's office; it also reviews the literature that describes similar deaths.
Abstract
The heterozygous condition characterized by the presence of hemoglobin AS (sickle-cell trait) occurs in approximately 8 percent of the American black population. Unlike the homozygous state (sickle-cell disease), sickle-cell trait is not widely recognized as a cause of life-threatening illness or death despite over 30 case reports that describe fatal or serious complications of exercise in young black males with this condition. These reports identify heat stress, dehydration, viral illness, and poor physical conditioning as factors that may contribute to exertional rhabdomyolysis and sudden death, suggesting multifactorial etiology; however, since sickling is known to occur postmortem, it remains controversial as to whether the pathogenesis of these exercise-related deaths involve microvascular obstruction by sickled erythrocytes. The authors describe three young black individuals with no significant past medical history who died following physical exertion. In all three cases, postmortem hemoglobin electrophoresis demonstrated hemoglobin AS. In none of the cases was the body temperature found to be elevated. These cases should remind the forensic community that, in the proper setting, sickle-cell trait must be viewed as a potentially fatal disorder. 3 figures and 10 references