Hafeez Farkhanda, Ahmad Tahir Masood, Anwar Sumair
Department of Paediatric Nephrology, The Children's Hospital and Institute of Child Health, Lahore.
J Coll Physicians Surg Pak. 2005 Jun;15(6):329-32.
To determine the efficacy of three different treatment protocols in steroid resistant idiopathic nephrotic syndrome, (SRINS).
Interventional study.
Department of Nephrology at The Children's Hospital, Lahore, over a period of 3 years from January 2000 to December 2002.
Nephrotic children who did not respond to four weeks of steroid therapy (60mg/M2) followed by three pulses of methyl prednisolone (1Gm/1.73M2) over a period of one week were labeled as steroid resistant. Those with histopathological lesions of minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS) and Mes.PGN were divided into 3 groups. Group-I was treated with cyclophosphamide (CPM) and oral steroids, group-II with cyclosporine and oral steroids and group-III with pulse methyl prednisolone (MPP) and oral steroid + CPM. The response to treatment and course of disease were observed in each group.
Twenty patients with mean age of 4.4 years were enrolled. On the whole 10 (50%) had complete remission. In group-I, 5 (50%), in group-II, 3 (75%) and in group-III, 2 (33.3%) had complete remission. Depending upon histological lesion 100% (n=2) with MCD, 50% (n=6) with Mes.PGN and 25% (n=1) with FSGS achieved complete remission. Cyclosporine and CPM induced remission in 100% of patients with MCD, while in Mes.PGN response rate in group-I, II, and III was 100% (n=1), 50%(n=1), and 44.4%(n=4) respectively. In patients with FSGS, MPP was the only drug used with limited response of 25% (n=1).
Cyclosporine proved to be a better option for MCD and Mes.PGN, while MPP showed limited response in patients with FSGS.
确定三种不同治疗方案对激素抵抗型特发性肾病综合征(SRINS)的疗效。
干预性研究。
拉合尔儿童医院肾病科,从2000年1月至2002年12月,为期3年。
对接受为期四周的激素治疗(60mg/M2)且随后在一周内接受三次甲泼尼龙冲击治疗(1Gm/1.73M2)后无反应的肾病患儿标记为激素抵抗型。将具有微小病变病(MCD)、局灶节段性肾小球硬化(FSGS)和系膜增生性肾小球肾炎(Mes.PGN)组织病理学病变的患儿分为3组。第一组用环磷酰胺(CPM)和口服激素治疗,第二组用环孢素和口服激素治疗,第三组用甲泼尼龙冲击治疗(MPP)加口服激素 + CPM。观察每组的治疗反应和病程。
共纳入20例平均年龄4.4岁的患者。总体上10例(50%)完全缓解。第一组5例(50%),第二组3例(75%),第三组2例(33.3%)完全缓解。根据组织学病变,MCD患者100%(n = 2)、Mes.PGN患者50%(n = 6)、FSGS患者25%(n = 1)实现完全缓解。环孢素和CPM使100%的MCD患者缓解,而在Mes.PGN中,第一组、第二组和第三组的缓解率分别为100%(n = 1)、50%(n = 1)和44.4%(n = 4)。在FSGS患者中,MPP是唯一使用的药物,缓解率有限,为25%(n = 1)。
环孢素被证明对MCD和Mes.PGN是更好的选择,而MPP在FSGS患者中显示出有限的反应。