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Year : 2020  |  Volume : 30  |  Issue : 6  |  Page : 370--376

Clinico-pathological profile and outcome of C-3 Glomerulopathy in Indian Children

Richa Prakash1, Uma S Ali1, Alpana Ohri1, Shashank Nitin Parekhji1, Atul Deokar1, Shaila Khubchandani2 
1 Division of Pediatric Nephrology, Department of Pediatrics, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
2 Department of Pathology, Jaslok Hospital and MedicalResearch Institute, Mumbai, Maharashtra, India

Correspondence Address:
Richa Prakash
1A/1503, Green Acres, Lokhandwala Complex, Andheri (w), Mumbai - 400 053, Maharashtra
India

Introduction: There is paucity of data of C3 glomerulopathy in Indian children. Methods: First Indian pediatric case series where consecutive renal biopsies done over a period of ten years were reviewed to identify those patients who had isolated or predominant C3 deposits on immunofluorescent microscopy, fulfilling the criteria for C-3 glomerulopathy. The clinical, biochemical, serological, histopathological profile, eGFR and the need for renal replacement therapy was analyzed. Results: Eighteen patients, comprising 5.3% (18/298) of all renal biopsies, had C3 glomerulopathy, four with Dense Deposit Disease (DDD) and fourteen with C3 Glomerulonephritis (C3GN) with a median follow-up of 38.2 months. Median age of presentation was 7.45±3.03 years (2.5yrs- 13.5yrs) with nine boys and nine girls. Presentation was nephrotic syndrome in seven (39%), acute nephritic syndrome in three (16.7%), hematuria in five (27.7%) and acute kidney injury in three (16.7%). Median eGFR was 69 ml/min/1.73m2 (8.2-107 ml/min/1.73m2). Hematuria was seen in 16 (88%), proteinuria in 18 (100%) and low C3 in 16 (88%) at the time of presentation. Mesangioproliferative glomerulonephritis was the predominant pattern in DDD while C3GN showed a mix of mesangioproliferative, membranoproliferative, endocapillary and crescentic GN (p = 0.43).Complete or partial remission was seen in seven patients who received long term alternate day steroids alone or with added mycophenolate mofetil. The cumulative patient survival was 70.8%. Kaplan Meir analyses for renal survival without progression to ESRD was 60.2% at one year and 48.1% at five and ten years. Conclusion: Interstitial fibrosis and tubular atrophy on renal biopsy was an independent predictor of adverse renal outcome in the cohort (p = 0.013, HR8.1;95% CI -1.6-42).

How to cite this article:
Prakash R, Ali US, Ohri A, Parekhji SN, Deokar A, Khubchandani S. Clinico-pathological profile and outcome of C-3 Glomerulopathy in Indian Children.Indian J Nephrol 2020;30:370-376

How to cite this URL:
Prakash R, Ali US, Ohri A, Parekhji SN, Deokar A, Khubchandani S. Clinico-pathological profile and outcome of C-3 Glomerulopathy in Indian Children. Indian J Nephrol [serial online] 2020 [cited 2021 Jan 17 ];30:370-376
Available from: https://www.indianjnephrol.org/article.asp?issn=0971-4065;year=2020;volume=30;issue=6;spage=370;epage=376;aulast=Prakash;type=0