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- Title
Membranous nephropathy in Schimke immuno-osseous dysplasia.
- Authors
Özdemir, Nihal; Alpay, Harika; Bereket, Abdullah; Bereket, Gamze; Bıyıklı, Neşe; Aydoğan, Metin; Çakalağoğlu, Fulya; Kılıçaslan, Işın; Akpınar, İhsan
- Abstract
Schimke immuno-osseous dysplasia is a rare autosomal recessive multi-system disorder, with clinical features of growth retardation, spondylo-epiphyseal dysplasia, nephrotic syndrome and immunodeficiency beginning in childhood. Here, we report a new case, in a 10-year-old boy with characteristic symptoms of Schimke immuno-osseous dysplasia. The patient presented with short stature and, later, developed nephrotic syndrome and peritonitis. In addition, he had perinuclear anti-neutrophilic cytoplasmic antibody (p-ANCA)-positive arthritis. Renal pathology of the patients with this disease usually show focal segmental glomerulonephritis, whereas our patient had membranous nephropathy, which has not previously been reported.
- Subjects
NEPHROTIC syndrome in children; KIDNEY diseases; CELLULAR pathology; PEDIATRIC nephrology; NEPHROLOGY
- Publication
Pediatric Nephrology, 2006, Vol 21, Issue 6, p870
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-006-0082-x