儿童原发性肾病综合征病理类型与激素疗效探讨

Investigation of histological types in childhood primary nephrotic syndrome and effect of prednisone therapy

目的 :为了探讨儿童原发性肾病综合征病理类型与激素疗效之间的关系。 方法 :对 16年来经肾活检明确病理类型的 410例原发性肾病综合征患儿应用泼尼松治疗的疗效分析 ,初始剂量为 2 m g/( kg· d) ,最大量不超过6 0 mg/d,共用 8周 ,部分迟发敏感的患儿再延长 2周 ;如完全缓解则逐渐减量 ;如部分缓解或不缓解 ,则逐渐减量并加用免疫抑制剂。 结果 :410例中 ,微小病变 ( MCNS) 71例 ,系膜增生 ( Ms PGN) 2 5 5例 ,膜增殖 ( MPGN) 2 9例 ,膜性肾病 ( MN) 2 8例 ,局灶节段性肾小球硬化症 ( FSGS) 2 7例 ,对激素治疗完全效应者分别为 91.5 %、79.2 %、2 4.1%、2 1.4%和 2 2 .2 % ,后续治疗仍可取得部分疗效。 结论 :1对儿童原发性肾病综合征必须尽快明确病理类型 ;2病理类型为 MCNS和 Ms PGN者 ,宜正规、足量地使用泼尼松 ,以达到较好的治疗目的 ;3病理类型为 MN、FSGS和 MPGN者 ,亦宜足量应用 ,即使在足量应用期间疗效不高 。

Objectives: To investgate the relationship between histological types in childhood primary nephrotic syndrome and effect of prednisone therapy. Methods: Four hundred and ten primary nephrotic syndrome patients whose pathological diognosis were established by renal biopsy in the past 16 years were analysed retrospectively in details.All patients were treated with prednisone for 8 weeks,the initial dosage were 2 mg/(kg·d) . Some laterespond patients prolonged the theray for another 2 weeks, the dosage were reduced gradually after complete remmision,immunosupressive agents were added for partial remmision and no remmision patients. Results: In all 410 patients, 55 patients were minimal changes(MCNS) , 255 patients were mesangial proliferative glomerulonephritis(MsPGNS),28 patients were membranous nephropathy(MN) 29 patients were membranous proliferative glomerulonephritis(MPGN), 27 patients were focal segmental glomerulosclerosis( FSGS) , the percenage of complete response to prednisone were 91.5%,79.2%,24.1%,21.4% and 22.2% respectively. Some patients responded in prolonged therapy. Conclusions:① Renal biopasy must be done as soon as possible for childhood primary nephrotic syndrome;②To get better effect of therapy, prednisone should be used in full dosages with MCNS and MsPGN; ③Prolonged therapy of prednisone were important for MN,FSGS and MPGN patients.