Department of Nephrology, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Kong Jiang Road, 1665, 200092, Shanghai, China.
Department of Nephrology and Rheumatology, Children's Hospital of Shanghai, Shanghai Jiao Tong University, Shanghai, China.
Pathol Res Pract. 2019 May;215(5):952-956. doi: 10.1016/j.prp.2019.01.046. Epub 2019 Feb 1.
The identification of the M-type phospholipase A2 receptor (PLA2R) is a breakthrough recognized as a major target for adults with idiopathic membranous nephropathy (IMN). However, the role PLA2R played in pediatric patients with IMN, particularly in Chinese, has yet to be determined.
This retrospective study included 187 adult patients and 38 pediatric patients aged 17 years or younger with biopsy proved IMN. The pediatric cohort consisted of 27 children aged from 1 to 12 years and 11 children aged from 13 to 17. Glomerular expression of PLA2R was analyzed in stored, formalin-fixed, paraffin-embedded kidney biopsy sections.
PLA2R staining in glomerular deposits was observed in 82.7% and 42.1% of adult and pediatric patients with IMN, respectively. The PLA2R-positive staining patients with IMN presented with more severe clinical features than PLA2R-negative staining patients in both adult and pediatric cohorts. When compared to the young children patients with IMN, the adolescents exhibited a higher positive rate of PLA2R staining (81.8% versus 25.9%), similar to the adult patients.
The clinical features and prevalence of PLA2R positive staining in adolescent patients with IMN were similar to adult patients, suggesting that they probably have a close etiology and pathogenesis. However, most of the young children patients with IMN were PLA2R negative staining, suggesting a different underlying etiology.
M 型磷脂酶 A2 受体（PLA2R）的鉴定被认为是特发性膜性肾病（IMN）成人患者的主要靶点，这是一项突破性的发现。然而，PLA2R 在儿科 IMN 患者中的作用，尤其是在中国人群中的作用，尚未确定。
本回顾性研究纳入了 187 例成年患者和 38 例年龄在 17 岁及以下的经活检证实的 IMN 儿科患者。儿科患者组包括 27 例 1 至 12 岁儿童和 11 例 13 至 17 岁儿童。在储存的福尔马林固定、石蜡包埋的肾活检切片中分析 PLA2R 在肾小球中的表达。
在分别患有 IMN 的成年和儿科患者中，PLA2R 染色在肾小球沉积物中分别观察到 82.7%和 42.1%。PLA2R 阳性染色的 IMN 患者在成年和儿科患者队列中均表现出比 PLA2R 阴性染色患者更严重的临床特征。与 IMN 的年幼儿童患者相比，青少年患者的 PLA2R 染色阳性率更高（81.8%比 25.9%），与成年患者相似。
青少年 IMN 患者的临床特征和 PLA2R 阳性染色率与成年患者相似，提示它们可能具有密切的病因和发病机制。然而，大多数 IMN 的年幼儿童患者 PLA2R 染色阴性，提示潜在病因不同。
