Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome

Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome

Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder characterized by postaxial polydactyly, retinitis pigmentosa, central obesity, mental retardation, hypogonadism, and renal involvement. Renal involvement in various forms has been seen in BBS. Cases with nephrotic range proteinuria n...

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Main Authors: K K Singh, R Kumar, J Prakash, A Krishna
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2015-01-01
Series:Indian Journal of Nephrology
Subjects:
Bardet-Biedl syndrome
minimal change nephrotic syndrome
renal anomalies
Online Access:http://www.indianjnephrol.org/article.asp?issn=0971-4065;year=2015;volume=25;issue=5;spage=300;epage=302;aulast=Singh
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spelling doaj-082eec8473c5417db60addc9094cdd982020-11-24T21:03:44ZengWolters Kluwer Medknow PublicationsIndian Journal of Nephrology0971-40651998-36622015-01-0125530030210.4103/0971-4065.151765Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndromeK K SinghR KumarJ PrakashA KrishnaBardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder characterized by postaxial polydactyly, retinitis pigmentosa, central obesity, mental retardation, hypogonadism, and renal involvement. Renal involvement in various forms has been seen in BBS. Cases with nephrotic range proteinuria not responding to steroid have been described in this syndrome. Here we report a case of BBS who presented with nephrotic range proteinuria. The biopsy findings were suggestive of minimal change disease. The child responded well to steroid therapy and remains in remission.http://www.indianjnephrol.org/article.asp?issn=0971-4065;year=2015;volume=25;issue=5;spage=300;epage=302;aulast=SinghBardet-Biedl syndromeminimal change nephrotic syndromerenal anomalies
collection DOAJ
language English
format Article
sources DOAJ
author K K Singh
R Kumar
J Prakash
A Krishna
spellingShingle K K Singh
R Kumar
J Prakash
A Krishna
Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome
Indian Journal of Nephrology
Bardet-Biedl syndrome
minimal change nephrotic syndrome
renal anomalies
author_facet K K Singh
R Kumar
J Prakash
A Krishna
author_sort K K Singh
title Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome
title_short Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome
title_full Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome
title_fullStr Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome
title_full_unstemmed Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome
title_sort bardet-biedl syndrome presenting with steroid sensitive nephrotic syndrome
publisher Wolters Kluwer Medknow Publications
series Indian Journal of Nephrology
issn 0971-4065
1998-3662
publishDate 2015-01-01
description Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder characterized by postaxial polydactyly, retinitis pigmentosa, central obesity, mental retardation, hypogonadism, and renal involvement. Renal involvement in various forms has been seen in BBS. Cases with nephrotic range proteinuria not responding to steroid have been described in this syndrome. Here we report a case of BBS who presented with nephrotic range proteinuria. The biopsy findings were suggestive of minimal change disease. The child responded well to steroid therapy and remains in remission.
topic Bardet-Biedl syndrome
minimal change nephrotic syndrome
renal anomalies
url http://www.indianjnephrol.org/article.asp?issn=0971-4065;year=2015;volume=25;issue=5;spage=300;epage=302;aulast=Singh
work_keys_str_mv AT kksingh bardetbiedlsyndromepresentingwithsteroidsensitivenephroticsyndrome
AT rkumar bardetbiedlsyndromepresentingwithsteroidsensitivenephroticsyndrome
AT jprakash bardetbiedlsyndromepresentingwithsteroidsensitivenephroticsyndrome
AT akrishna bardetbiedlsyndromepresentingwithsteroidsensitivenephroticsyndrome
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