An Atypical Nephrotic Syndrome


Article PDF :

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Article type :

Case report

Author :

Shetty S.,* Popat I.,*** Kadam NN.,** Viswanathan V.,# Nikhil B.,* Ragi R.*

Volume :

5

Issue :

2

Abstract :

A 14 year old girl presented with peri-orbital puffiness of face, pedal oedema and decreased urine output for 2 weeks. She had similar history with hematuria 3 years back. On examination, she had generalized anasarca, pallor, faint malar rash, swollen and painful knees, hypertension and ascites. Earlier investigations had shown microalbuminuria, creatinine 1.7, urine albumin: creatinine ratio 560, ANA and dsDNA both positive, low C3 and high serum cholesterol. Ascitic fluid was sterile. The child had then received prednisolone but no resolution even after 6 weeks, followed by 7 cycles of IV cyclophosphamide. She was re-evaluated as an atypical nephrotic with SLE like features and a kidney biopsy taken. With the histopathological diagnosis of Lupus nephritis type V, she was put on appropriate therapy as per ACR, 2011 guidelines and showed good response. The current management guidelines of Lupus Nephritis discussed.

Keyword :

lupus nephritis, atypical nephrotic syndrome, ACR guidelines
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