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目的 探讨婴幼儿期原发性肾病综合征 (简称婴幼儿肾病 )的临床特点 ;分析婴幼儿肾病临床特点、免疫功能、病理分型和糖皮质激素 (简称激素 )疗效的关系。方法 对 31例婴幼儿肾病患儿进行临床观察 ;进行体液免疫和细胞免疫功能测定 ;14例接受肾穿刺活检 ;31例均采用激素中长程疗法 ,18例予以免疫抑制剂如环磷酰胺 (CTX)等联合治疗。结果 婴幼儿肾病临床以肾炎型肾病为主 ;体液免疫和细胞免疫功能下降 ;病理以非微小病变型为主 ;约 6 0 %患儿对激素治疗不敏感 ,需用激素与免疫抑制剂联合治疗。结论 婴幼儿肾病具有与其它儿童肾病综合征不同的特点 ,应当引起临床重视。
Objective To investigate the clinical features of primary nephrotic syndrome in infancy and early childhood and to analyze the clinical features, immune function, pathological classification and the effect of glucocorticoid (Hormone) in infants and young children with nephropathy. Methods Thirty-one infants and young children with nephropathy were enrolled in this study. The humoral and cellular immune function tests were performed. Fourteen patients underwent renal biopsy. Thirty-one patients were treated with mid-to-long-term hormone therapy and immunosuppressive agents such as cyclophosphamide ) And other combination therapy. Results Nephrotic nephropathy was mainly nephritic nephropathy in infants and young children. The humoral and cellular immune functions were decreased. The pathological features were non-minimal lesions. About 60% of the children were not sensitive to hormonal therapy. Hormone and immunosuppressive agents were needed . Conclusions Infantile nephropathy has different characteristics from other children with nephrotic syndrome, which should cause clinical attention.