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Steroid-responsive nephrotic syndrome in a patient with nail-patella syndrome

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Abstract

Nail-patella syndrome (NPS) is a rare disorder with autosomal dominant mode of inheritance. We report a child with NPS and steroid-responsive, frequently relapsing nephrotic syndrome. The child had dystrophic nails, flexion contractures of both elbows and normal renal functions. X-rays of the knees and pelvis showed hypoplastic patellae and iliac horns. Renal histology was unremarkable with mild focal increase in mesangial cellularity compatible with minimal change disease. Ultrastructural features of NPS including thickening of the glomerular basement membrane with electron-lucent areas were not found.

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Authors and Affiliations

  1. Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India

    Pankaj Hari, Mukta Mantan & Arvind Bagga

  2. Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India

    Amit Dinda

  3. Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India

    Smriti Hari

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  1. Pankaj Hari
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  2. Mukta Mantan
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  3. Amit Dinda
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  4. Smriti Hari
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  5. Arvind Bagga
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Correspondence to Arvind Bagga.

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Hari, P., Mantan, M., Dinda, A. et al. Steroid-responsive nephrotic syndrome in a patient with nail-patella syndrome. Pediatr Nephrol 21, 1197–1199 (2006). https://doi.org/10.1007/s00467-006-0154-y

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  • DOI: https://doi.org/10.1007/s00467-006-0154-y

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