Steroid-responsive nephrotic syndrome in a patient with nail-patella syndrome.
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.
Hari
Pankaj
P
Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.
Mantan
Mukta
M
Dinda
Amit
A
Hari
Smriti
S
Bagga
Arvind
A
D002648:Child; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009261:Nail-Patella Syndrome / Q000150:complications*; D009404:Nephrotic Syndrome / Q000150:complications* / Q000188:drug therapy*; D011239:Prednisolone / Q000627:therapeutic use*; D012008:Recurrence; D012074:Remission Induction
D005938:Glucocorticoids; D011239:Prednisolone