儿童膜增生性肾炎5例临床病理分析

Clinical and pathological characteristics of membranoproliferative glomerulonephritis:A report of 5 cases

摘要:

目的：进一步提高对膜增生性肾炎的病理及临床的认识,探讨治疗方法。方法：对5例病理诊断为膜增生性肾炎患儿的病理、临床表现特点、治疗转归进行分析。结果：①5例临床表现为肾病水平的蛋白尿及血尿, 4例有高血压, 2例肾功不全;②病理改变肾小球系膜细胞增生及系膜基质扩张, 3例肾小球呈分叶状, 2例有新月体形成, 4例基膜“双轨征”,肾间质有不同程度的损害,免疫荧光均以C3 沉积为主;③5例均应用了泼尼松治疗,其中3例应用了甲基泼尼松龙冲击, 4例联合应用了环磷酰胺静脉冲击治疗。3例获部分缓解, 1例完全缓解, 1例呈持续肾病状态。结论：儿童膜增生性肾小球肾炎临床表现以肾炎型肾病为主。病理以系膜细胞增生基质扩张和基膜“双轨征”为其特征性改变,免疫荧光检查以C3沉积为主。大剂量激素联合环磷酰胺静脉冲击治疗对短期内缓解病情、改善肾功能是有效的。

Abstract:

OBJECTIVE: To understand the clinical and pathological characteristics of the membranoproliferative glomerulonephritis (MPGN) and to explore the therapeutic methods. METHODS: The clinical characteristics, laboratory data, pathological findings and therapeutic methods were investigated retrospectively in the 5 patients with MPGN (aged 8-13 years). RESULTS:  ①All five patients presented with nephritic-range proteinuria (>50 mg/kg· d) and haematuria, 4 patients had hypertension and low serum C_3 levels; and 2 patients, azotemia. ②Renal biopsies revealed mesangial hypercellularety and expanded mesangial matrix in 5 cases, glomerular lobulation, in 3 cases, and crescent formation, in 2 cases. The double contour of basement membrane occurred in 4 cases. Different degrees of tubulointerstitial damage occurred in 5 cases. Immunofluoroscence showed C_3 deposition in 5 cases. ③ All the 5 patients received prednisone combined with intravenous cyclophosplamide (CTX ) alone or added methyprednisolone together with pulse therapy. After treatment, 1 patient achieved a complete remission; 3, partial remission, and persistent nephropathy in 1 case. CONCLUSIONS: The study suggested that clinical manifestation in children with MPGN is characterized by nephritic nephrosis. The pathological changes are characterized by mesangial cells proliferation, expanded mesangial matrix and double contour of basement membrance. High dosage corticosteroids combined with CTX pulse therapy may be effective for improving the short outcome of MPGN.