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目的探讨儿童特发性膜性肾病(IMN)的临床和病理特征。方法 1999年7月至2009年7月在中山大学附属第一医院确诊的IMN患儿13例,回顾性分析其临床病理特征、治疗及转归情况。结果 13例IMN患儿中男11例,女2例;就诊时中位年龄为11.4(3.0～14.5)岁;肾穿时中位病程为3.7(1.4～65.3)个月。确诊时临床表现为肾病综合征(NS)9例(单纯型1例,肾炎型8例),血尿蛋白尿3例,复发性肉眼血尿1例,伴高血压1例(7.7%)。所有患儿的肾功能均正常。肾脏病理结果:(1)光镜病理分期:Ⅰ期4例,Ⅱ期9例。4例伴肾小球球性硬化(球性硬化肾小球百分比分别为11.8%、2.9%、7.5%、4.5%),1例伴节段性硬化(节段硬化肾小球百分比为4.5%),3例伴肾小管萎缩,1例伴细胞纤维性新月体形成(3.8%)。(2)免疫荧光:以IgG、C3在肾小球基底膜(GBM)上皮下沉积为主(前者12/12例、后者10/12例),少数伴IgM、Fg、C1q和IgA沉积,但沉积强度较IgG、C3弱。(3)电镜:13例患儿GBM均有不同程度增厚,2例毛细血管腔内出现微血栓。根据患儿临床表现及病理分期予激素和(或)免疫抑制剂治疗,联用或单用血管紧张素转化酶抑制剂/血管紧张素Ⅱ受体拮抗剂。9例NS患儿中,完全缓解(CR)5例,部分缓解(PR)1例,无效3例(其中2例患儿改用霉酚酸酯治疗获CR,1例加用大剂量丙种球蛋白冲击治疗后获CR);3例血尿蛋白尿患儿中,1例尿蛋白转阴,但仍有持续性镜下血尿,2例失访;1例复发性肉眼血尿患儿治疗后肉眼血尿消失,但仍有镜下血尿。结论儿童IMN多发于学龄期男性,临床表现以肾炎型NS为主。病理分期以Ⅰ、Ⅱ期多见,免疫荧光以IgG、C3在GBM上皮下沉积为主,电镜示所有患儿GBM均有不同程度增厚。目前国内儿童IMN尚无统一治疗方案,亟需在临床实践中加强随访及注重个体化治疗,以提高疗效,改善预后。
Objective To investigate the clinical and pathological features of idiopathic membranous nephropathy (IMN) in children. Methods Thirteen patients with IMN diagnosed in the First Affiliated Hospital of Sun Yat-sen University from July 1999 to July 2009 were retrospectively analyzed for their clinicopathological characteristics, treatment and outcome. Results There were 11 males and 2 females with IMN in 13 cases. The median age at visit was 11.4 (3.0-14.5) years. The median duration of renal wear was 3.7 (1.4-65.3) months. The clinical manifestations at the time of diagnosis were nephrotic syndrome (NS), 9 (1 simple type, 8 nephritis type), 3 urine proteinuria, 1 recurrent macroscopic hematuria, and 1 patient (7.7%) with hypertension. All children had normal kidney function. Renal pathological results: (1) light microscopy staging: Ⅰ in 4 cases, Ⅱ in 9 cases. 4 cases had glomerular sclerosis (the percentage of glomerular sclerosis was 11.8%, 2.9%, 7.5%, 4.5% respectively), and 1 patient had segmental sclerosis (the percentage of sclerosis glomeruli was 4.5% ), 3 with tubular atrophy and 1 with fibrocystic crescent formation (3.8%). (2) Immunofluorescence: IgG and C3 were mainly subcutaneously deposited on the glomerular basement membrane (GBM) (the former 12/12 cases and the latter 10/12 cases). A few of them were deposited with IgM, Fg, C1q and IgA. However, the deposition intensity of IgG, C3 is weak. (3) Electron microscopy: Thirteen children with GBM had varying degrees of thickening, and two cases of capillary thrombosis in the capillary cavity. According to the clinical manifestations and pathological staging of hormone and / or immunosuppressive therapy, combined with or alone with angiotensin converting enzyme inhibitor / angiotensin Ⅱ receptor antagonist. Among the 9 children with NS, 5 were completely relieved (CR), 1 was partly relieved (PR) and 3 was ineffective (2 of them were treated with mycophenolate mofetil, and 1 was treated with high dose of gamma bulbs Proteinuria after CR treatment); 3 cases of urine proteinuria in children, 1 urine protein negative, but there is still persistent microscopic hematuria, 2 cases were lost to follow-up; 1 case of relapsed patients with gross hematuria gross hematuria Disappear, but still microscopic hematuria. Conclusion Childhood IMN is more common in school-age men and its clinical manifestations are nephritis-type NS. The pathological staging was more common in stage Ⅰ and Ⅱ, and the immunofluorescence was mainly based on the deposition of IgG and C3 in GBM. Electron microscopy showed that GBM of all children had different degrees of thickening. At present, there is no unified treatment plan for domestic children IMN, so it is urgent to strengthen follow-up and individualized treatment in clinical practice in order to improve curative effect and improve prognosis.