Kumar Vinod, Varma Ashwani Kumar, Nada Ritambhra, Ghosh Ratan, Suri Deepti, Gupta Anju, Kumar Vivek, Rathi Manish, Kohli Harbir, Jha Vivekanand, Gupta Krishan, Ramachandran Raja
Department of Nephrology, Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Histopathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Nephrology (Carlton). 2017 Sep;22(9):678-683. doi: 10.1111/nep.12835.
Primary membranous nephropathy (PMN) accounts for only 1-2% of nephrotic syndrome in children. Antibodies to m-type phospholipase A2 receptor (aPLA R) is seen in 70% of adult PMN cases. The present study was undertaken to study m-type phospholipase A receptor (PLA R) status and clinical behavior in adolescent PMN cases.
The present prospective observational study included adolescent (10-19 years) onset biopsy proved PMN. Patients were followed on a monthly basis with urine protein, serum albumin and creatinine. Serum aPLA R was done at baseline and at 6 and 12 months of starting treatment. Patients were treated as per unit's protocol.
During the study period a total of 18 patients were enrolled. The mean age of the cases was 16.27 ± 2.39 (11-19) years. Seventeen (94.44%) patients presented with nephrotic syndrome. The mean proteinuria and serum albumin was 4.52 ± 1.93 (2.43-9.20) g/day and 2.1 ± 0.6 (1.1-3.4) g/dL respectively. PMN was PLA R related in 83%. aPLA2R and enhanced staining for PLA R in glomeruli was seen in 14 (77.78%) and 13 (72.22%), respectively. Clinical remission at the end of 6 and 12 months of therapy was seen in 11 (61.11%) and 9 (50%) subjects respectively. There was a significant association of aPLA R to clinical remission/ resistance.
Primary membranous nephropathy in adolescent population is aPLA R related in over three-quarters of the cases and the response to therapy is seen in only half of them. aPLA R monitoring is clinically relevant and should be incorporated in the management of adolescent onset PMN.
原发性膜性肾病（PMN）在儿童肾病综合征中仅占1%-2%。70%的成人PMN病例中可检测到抗m型磷脂酶A2受体（aPLA R）抗体。本研究旨在探讨青少年PMN病例中m型磷脂酶A受体（PLA R）的状态及临床特征。
本前瞻性观察性研究纳入了经活检证实为青少年（10-19岁）起病的PMN患者。每月对患者进行尿蛋白、血清白蛋白和肌酐检测。在基线、开始治疗6个月和12个月时检测血清aPLA R。患者按照科室方案进行治疗。
研究期间共纳入18例患者。病例的平均年龄为16.27±2.39（11-19）岁。17例（94.44%）患者表现为肾病综合征。平均蛋白尿和血清白蛋白分别为4.52±1.93（2.43-9.20）g/天和2.1±0.6（1.1-3.4）g/dL。83%的PMN与PLA R相关。分别有14例（77.78%）和13例（72.22%）患者的肾小球中检测到aPLA2R及PLA R染色增强。治疗6个月和12个月时，分别有11例（61.11%）和9例（50%）患者实现临床缓解。aPLA R与临床缓解/抵抗显著相关。
青少年原发性膜性肾病中超过四分之三的病例与aPLA R相关，且只有一半的患者对治疗有反应。aPLA R监测具有临床相关性，应纳入青少年起病PMN的管理中。
