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儿童Ⅰ型膜增生性肾小球肾炎的长期皮质类固醇和双嘧达莫治疗

Long-term corticosteroid and dipyridamole treatment of membranoproliferative glomerulonephritis type I in children.

作者信息

Takeda A, Niimura F, Matsutani H

机构信息

Department of Pediatrics, School of Medicine, Tokyo Medical and Dental University, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 1995 Jun;37(6):330-5.

PMID:7666598
Abstract

Eighteen patients with biopsy-proven membranoproliferative glomerulonephritis (MPGN) type I (16 diffuse, 2 focal) and a minimum of three years of follow-up (median 6.0 years, range 3 to 15.5 years) were treated with corticosteroids combined with dipyridamole. Initial therapy consisted of intravenous methylprednisolone pulses and/or daily oral prednisolone at the dose of 1 mg/kg combined with dipyridamole at the dose of 5 mg/kg, depending on the disease severity. Corticosteroid dosage was then reduced according to clinical improvement. At the start of therapy, 12 patients had microscopic hematuria or mild proteinuria, three patients had heavy proteinuria and three patients had insufficient renal function. At a median of 53 months of treatment, 89% of the children had normal or mildly abnormal urinary findings with normal renal function. Follow-up biopsy revealed improved active glomerular injury in all children. Although one case progressed to end-stage renal failure, the renal survival of our patients was 94% at 4 to 10 years after clinical onset. No patient suffered a relapse during corticosteroid dosage adjustment, nor did any unacceptable complications arise during the regimen. Eleven patients stopped the regimen because their renal function and urinalysis had normalized, and they remained in remission for a median of 32 months. These results suggest that long-term, tapering and limited use of corticosteroids combined with dipyridamole is useful and safe for children with MPGN type I.

摘要

18例经活检证实为I型膜增生性肾小球肾炎(MPGN)(16例弥漫性、2例局灶性)且随访至少3年(中位随访时间6.0年,范围3至15.5年)的患者接受了皮质类固醇联合双嘧达莫治疗。初始治疗包括静脉注射甲泼尼龙冲击治疗和/或每日口服泼尼松龙,剂量为1mg/kg,联合双嘧达莫,剂量为5mg/kg,具体取决于疾病严重程度。随后根据临床改善情况减少皮质类固醇剂量。治疗开始时,12例患者有镜下血尿或轻度蛋白尿,3例患者有大量蛋白尿,3例患者肾功能不全。在治疗中位时间53个月时,89%的儿童尿检查结果正常或轻度异常,肾功能正常。随访活检显示所有儿童的活动性肾小球损伤均有改善。虽然有1例进展为终末期肾衰竭,但我们患者的肾脏生存率在临床发病后4至10年为94%。在皮质类固醇剂量调整期间,没有患者复发,在治疗方案实施过程中也没有出现任何不可接受的并发症。11例患者停止治疗,因为他们的肾功能和尿液分析已恢复正常,并且他们的缓解期中位时间为32个月。这些结果表明,长期、逐渐减量并有限使用皮质类固醇联合双嘧达莫对I型MPGN儿童是有用且安全的。

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