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HISTIOCYTOID CARDOMYOPATHY: CASE REPORT AND LITERATURE REVIEW

NCJ Number
145908
Journal
Journal of Forensic Sciences Volume: 38 Issue: 6 Dated: (November 1993) Pages: 1427-1435
Author(s)
J A Prahlow; L A Teot
Date Published
1993
Length
9 pages
Annotation
This article presents a case study and a literature review that documents histiocytoid cardiomyopathy as a rare cause of sudden infant death.
Abstract
The sudden death of an infant may be due to a variety of causes. In such cases, complete autopsy with radiologic, gross, microscopic, and toxicological examination is warranted. This article presents the case of a previously healthy 15-month-old girl with no known disease, who experienced witnessed cardiac arrest, ventricular fibrilation, and death. Complete postmortem examination revealed histiocytoid cardiomyopathy as the cause of death. Histiocytoid cardiomyopathy is a rare infantial cardiac- muscle disorder characterized by an enlarged, polygonal subendocardial myocytes that, by light microscopy, reveal granular, faintly eosinophilic cytoplasm instead of the normal striations. Ultrastructurally, the myocytes contain many mitochondria and markedly reduced numbers of myofibrils. Clinically, the disorder has symptoms of cardiac arrhythimias and/or sudden death that occurs in children under 2 years old. This is a rare condition that typically affects females under 2 years old. It is apparently an inborn or acquired metabolic derangement within the mitochondria of certain myocytes. Since sudden death may be the initial presentation of the disorder, the forensic community should take this disorder into account in the investigation of sudden infant deaths. 5 figures and 24 references