| ORIGINAL ARTICLE |
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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
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijn.IJN_226_18
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).
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