CONGENITAL GENERALIZED LIPODYSTROPHY - REPORT ON ONE CASE, WITH SPECIAL REFERENCE TO POSTMORTEM FINDINGS

  • 1 January 1976
    • journal article
    • research article
    • Vol. 84  (1) , 47-54
Abstract
Generalized lipodystrophy (Berardinelli-Seip''s syndrome) was diagnosed in a boy at the age of 8.5 mo. Ten mo. later he died because of aspiration of food. Necropsy with special reference to the CNS revealed hypothalamic lesions, probably of a malformative or hamartomatous nature. The findings support the view that hypothalamic dysfunction is responsible for the signs and symptoms of generalized lipodystrophy.

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