RENAL AFFECTION IN HEREDITARY OSTEO-ONYCH ODYSPLASIA

Review of the literature and case report concern renal damage in hered itary osteo-onychodysplasia (nail-patella syndrome). This syndrome is a rare genetic disease inherited by the autosome-dominant type. It ari ses because of underdevelopment of tissues of mesodermal and ectoderma l origin and is characterized by hypoplasia or absence of patella, uln ar dysplasia, nail plate dystrophy, growths on the iliac bones, renal affection. The disease was detected in early childhood. Renal affectio n presented with urinary syndrome with a concurrent nephrotic syndrome later on followed by moderate arterial hypertension and progression t o renal failure. Renal biopsy re vealed the picture of focal segmentar y glomerular hyalinosis typical for this disorder. Because immunodepre ssants were thought inadequate, the treatment included lovastatin and inhibitors of angiotensin-converting enzyme to inhibit non-immune mech anisms of the disease progression.