The cardiac lesions in Bassen-Kornzweig syndrome: Report of a case, with autopsy findings

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The autopsy findings in a case of Bassen-Kornzweig's syndrome in which death was due to cardiac failure are recorded. Interstitial myocardial fibrosis and long tract degeneration in the spinal cord similar to that seen in fatal cases of Friedreich's ataxia were the chief cardiac and nervous system findings. Excessive deposition of lipochrome pigment in intestinal and cardiac muscle was a prominent feature. The implications of these findings and their relationship to faulty lipid and vitamin absorption from the intestine are discussed.

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From the Department of Pathology, Cornell University Medical Center, New York, New York 10021.

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