The Nephropathy of Hereditary Osteo-onychodysplasia

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Abstract
Hereditary osteo-onychodysplasia is a genetically dominant syndrome characterized chiefly by dysplasia of the fingernails, the presnece of iliac horns, hypoplastic or absent patellae and deformities of the head of the radius. In the medical literature, an associated renal lesion is obvious. A review of previously described cases shows a high incidence of proteinuria. One report described 6 members in a family with evidence of renal involvement manifested by proteinuria, abnormal urinary sediment and diminished renal function. Two patients in the family died with probable renal disease. Another author described the necropsy findings in a man with hereditary osteo-onycho-dysplasia who died in renal failure. The renal tissue suggested chronic glomerulonephritis. There is no ante-mortem description of renal tissue in this syndrome in the medical literature. Four members of a family with hereditary osteo-onychodysplasia were studied by the author.