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 CASE REPORT
Year : 2015  |  Volume : 25  |  Issue : 5  |  Page : 300-302

Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome


1 Department of Pediatrics, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
2 Department of Nephrology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India

Correspondence Address:
R Kumar
Department of Pediatrics, Indira Gandhi Institute of Medical Sciences, Patna, Bihar
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-4065.151765

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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.






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Indian Journal of Nephrology
Published by Wolters Kluwer - Medknow
Online since 20th Sept '07