HEREDITARY SCLEROSING GLOMERULOPATHY IN THE CONORENAL SYNDROME

Kidney failure is recognized to occur in association with bone malform ations, yet the renal disease often is incompletely characterized. In the syndrome of cone-shaped epiphyses of the phalanges and renal failu re (conorenal syndrome), the kidney disease has been previously labele d ''nephronophthisis'' (now termed ''medullary cystic disease''). We r eport two siblings with the conorenal syndrome in whom longitudinal cl inical study has been possible and from whom kidney biopsy specimens w ere obtained prior to renal failure; their renal disease is incompatib le with medullary cystic disease. The variable clinical course and nep hropathology of this syndrome are characterized. These results call in to question the association of medullary cystic disease of the kidney with other syndromes of bone dysplasia with renal failure. (C) 1995 by the National Kidney Foundation, Inc.