Prakash Richa, Ali Uma S, Ohri Alpana, Parekhji Shashank Nitin, Deokar Atul, Khubchandani Shaila
Division of Pediatric Nephrology, Department of Pediatrics, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India.
Department of Pathology, Jaslok Hospital and Medical Research Institute, Mumbai, Maharashtra, India.
Indian J Nephrol. 2020 Nov-Dec;30(6):370-376. doi: 10.4103/ijn.IJN_226_18. Epub 2020 Feb 7.
There is paucity of data of C3 glomerulopathy in Indian children.
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.
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/1.73m (8.2-107 ml/min/1.73m). 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.
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).
关于印度儿童C3肾小球病的数据较少。
首个印度儿科病例系列，回顾了十年间连续进行的肾活检，以确定那些在免疫荧光显微镜检查中存在孤立性或主要C3沉积、符合C3肾小球病标准的患者。分析了其临床、生化、血清学、组织病理学特征、估算肾小球滤过率（eGFR）以及肾脏替代治疗的需求。
18例患者患有C3肾小球病，占所有肾活检的5.3%（18/298），其中4例为致密物沉积病（DDD），14例为C3肾小球肾炎（C3GN），中位随访时间为38.2个月。发病时的中位年龄为7.45±3.03岁（2.5岁至13.5岁），男9例，女9例。表现为肾病综合征7例（39%），急性肾炎综合征3例（16.7%），血尿5例（27.7%），急性肾损伤3例（16.7%）。中位eGFR为69 ml/min/1.73m²（8.2 - 107 ml/min/1.73m²）。发病时16例（88%）有血尿，18例（100%）有蛋白尿，16例（88%）C3降低。系膜增生性肾小球肾炎是DDD的主要病理类型，而C3GN表现为系膜增生性、膜增生性、内皮细胞增生性和新月体性肾小球肾炎的混合类型（p = 0.43）。7例接受长期隔日使用类固醇单独治疗或联合霉酚酸酯治疗的患者实现了完全或部分缓解。患者累积生存率为70.8%。Kaplan - Meir分析显示，未进展至终末期肾病（ESRD）的肾脏生存率在1年时为60.2%，5年和10年时分别为48.1%。
肾活检中的间质纤维化和肾小管萎缩是该队列中不良肾脏结局的独立预测因素（p = 0.013，风险比8.1；95%可信区间 - 1.6 - 42）。
