38例儿童特发性IgM肾病临床与病理分析

Clinical Pathological Analysis of 38 Cases of IgM Nephropathy in Children

目的 探讨小儿IgM肾病的临床病理特点及预后。 方法 收集整理同济医院 38例IgM肾病患儿病例 ,对其临床表现、病理及疗效进行分析。结果 IgM肾病多表现为肾病综合征 (17例 ) ,其次为单纯性血尿 (13例 ) ,也可表现为持续性蛋白尿、血尿伴蛋白尿。 38例均可见IgM弥漫性沉积于肾小球系膜区 ,31例 (81 5 8% )可见电子致密物的沉积 ,2 0例 (5 2 6 3% )存在球囊粘连 ,9例 (2 3 6 8% )伴有间质炎性细胞浸润。 17例肾病综合征者经激素或激素加免疫抑制剂治疗后均可完全缓解。 13例单纯性血尿者经中药及对症治疗后 ,肉眼血尿消失 ,镜下血尿有不同程度的减轻但仍持续存在。随访 2 5例 ,2 1例复发 (84 % )但无患儿发生肾功能不全 ,其预后有待进一步观察。结论儿童IgM肾病有其独特的临床病理特征及预后 ,应将其作为一种独立的疾病 ,以便更好地指导临床治疗。

Objective To investigate the clinical pathological characters and prognosis of IgM nephropathy in children.Methods The data of 38 cases of IgM nephropathy in Tongji Hospital were reviewed. The clinical characters, pathological features and curative effects were analyzed. Results The main clinical manifestations of IgM nephropathy in children included nephritic syndrome (NS, 17 cases), isolated hematuria (13 cases), and others such as proteinuria, proteinuria combined with hematuria. IgM deposited diffusely in mesenteric area in all cases. Thirty-one cases (81.58 %) had dense deposit. Twenty cases (52.63%) had the adhesion between the glomerulus and glomerular capsule. Nine cases (23.68 %) had infiltration of intestitial inflammatory cells. After treatment with corticosteroid or corticosteroid in combination with immunosuppression, 17 NS children were completely remitted. Thirteen children with isolated hematuria received the traditional chinese medicine and other therapy according to their symptoms. Gross hematuria disappeared in all cases and microscopic hematuria relieved at varying degrees but lasted. In 25 receiving follow-up, 21 children relapsed, none had renal dysfunction and most had the better prognosis. Conclusion IgM nephropathy in children showed special clinical pathological features and prognosis. It should be seperated as an independent disease to direct therapy better.