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September 1966

The Hereditary Nephropathy of Osteo-onychodysplasia: Nail-Patella Syndrome

Author Affiliations

From the Department of Pathology, University of Michigan, Ann Arbor.

Am J Dis Child. 1966;112(3):237-241. doi:10.1001/archpedi.1966.02090120105011

OSTEO-ONYCHODYSPLASIA, often called nail-patella syndrome or by other eponymic designations,1 is a hereditary condition in which variable combinations of dysplasia of the nails, skeletal deformities involving the elbow joint, accessory pelvic posterior iliac horns, and anomolous development of the patella most commonly occur. Renal disease frequently constitutes a part of this syndrome, but death due to the hereditary nephropathy is unusual. The majority of patients with renal lesions are asymptomatic and manifest only proteinuria.1-3

A histopathological categorization of the renal lesion in this syndrome has not been clearly defined. The relatively benign clinical course of this renal lesion in most affected individuals must certainly contribute to the paucity of tissue specimens that have been studied.

The following case represents the second instance in which the antemortem renal pathology of this condition has been reported, and the only instance, as far as the author has determined, in which both

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