PEDIATRICS Vol. 64 No. 1 July 1979, pp. 24-29
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An X-Linked Recessive Cardiomyopathy with Abnormal Mitochondria

Harry B. Neustein MD1, Paul R. Lurie MD1, Beverly Dahms MD1, and Masato Takahashi MD1

1 Department of Pathology and Department of Pediatrics, Division of Cardiology, Childrens Hospital of Los Angeles, University of Southern California School of Medicine, Los Angeles

A transvascular endomyocardial biopsy from an infant with cardiomyopathy and chronic congestive heart failure showed abnormal mitochondria when examined by electron microscopy. At necropsy, similar abnormal mitochondria were seen in skeletal muscles, liver, and kidney. The patient's family pedigree revealed several male babies who had cardiac disease and died in infancy. Myocardium obtained at necropsy from three cousins contained mitochondria with abnormalities similar to those from the proband. An X-linked recessive cardiomyopathy seems likely in this family.

Submitted on April 10, 1978
Accepted on October 19, 1978




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