Abstract

Clinical and pathologic findings in a 13 13 12 year old girl with coronary artery disease of an apparently unique type are described. Necropsy revealed a generalized arterial disease characterized by degeneration and calcification of the media with focal thickening of the intima. Other findings included antinuclear antibodies, low serum copper and ceruloplasmin levels, anetoderma and pulmonary granulomas. These findings suggest the possibility of an underlying autoimmune or metabolic disorder.

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