title: Steroid-responsive nephrotic syndrome in a patient with nail-patella syndrome.
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.
authors.affiliation: Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.
authors.forename: Pankaj
authors.initials: P
authors.lastname: Hari
authors.forename: Mukta
authors.initials: M
authors.lastname: Mantan
authors.forename: Amit
authors.initials: A
authors.lastname: Dinda
authors.forename: Smriti
authors.initials: S
authors.lastname: Hari
authors.forename: Arvind
authors.initials: A
authors.lastname: Bagga
mesh_terms: 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
chemical_list: D005938:Glucocorticoids; D011239:Prednisolone