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  • Title: [Kidney involvement in hereditary osteo-onychodysplasia].
    Author: Kozlovskaia NL, Tareeva IE, Popova VV, Miroshnichenko NG, Stavrovskaia EV.
    Journal: Ter Arkh; 1997; 69(6):37-9. PubMed ID: 9297271.
    Abstract:
    Review of the literature and case report concern renal damage in hereditary osteo-onychodysplasia (nail-patella syndrome). This syndrome is a rare genetic disease inherited by the autosome-dominant type. It arises because of underdevelopment of tissues of mesodermal and ectodermal origin and is characterized by hypoplasia or absence of patella, ulnar dysplasia, nail plate dystrophy, growths on the iliac bones, renal affection. The disease was detected in early childhood. Renal affection presented with urinary syndrome with a concurrent nephrotic syndrome later on followed by moderate arterial hypertension and progression to renal failure. Renal biopsy revealed the picture of focal segmentary glomerular hyalinosis typical for this disorder. Because immunodepressants were thought inadequate, the treatment included lovastatin and inhibitors of angiotensin-converting enzyme to inhibit non-immune mechanisms of the disease progression.
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