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日本儿童特发性膜增生性肾小球肾炎

Idiopathic membranoproliferative glomerulonephritis in Japanese children.

作者信息

Iitaka K, Ishidate T, Hojo M, Kuwao S, Kasai N, Sakai T

机构信息

Department of Paediatrics, Kitasato University School of Medicine, Kanagawa, Japan.

出版信息

Pediatr Nephrol. 1995 Jun;9(3):272-7. doi: 10.1007/BF02254182.

Abstract

The course of idiopathic membranoproliferative glomerulonephritis (MPGN) in 41 Japanese children (21 boys, 20 girls) is reported. The mean follow-up period was 8 years, 9 months; 29 children with MPGN (71%) were identified by school urinary screening; 32 patients had type I MPGN, 2 type II and 7 type III; 10 patients were treated with multiple low-dose cocktail therapy (MLD), 8 with MLD followed by high-dose alternate-day (ALD) prednisolone and 21 with high-dose ALD prednisolone alone. In 1 patient, MPGN progressed to end-stage renal failure. The serum creatinine level in all of the remaining 40 patients was < or = 1.3 mg/dl at the last follow-up. Urinalysis was normal in 24 (59%). Of the 17 patients who still had urinary abnormalities, 4 had nephrotic syndrome. The incidence of remission of urinary abnormalities was highest in the patients treated with high-dose ALD prednisolone. Rebiopsy was performed in 33 patients, and revealed slight histological improvement in 11 (33%) patients, moderate improvement in 8 (24%), marked improvement in 5 (15%) and deterioration or no improvement in 9 patients (27%). Relatively few side effects of treatment were observed. The superior outcome of the MPGN patients in this compared with other studies may be the result of earlier detection and treatment.

摘要

报告了41名日本儿童(21名男孩,20名女孩)特发性膜增生性肾小球肾炎(MPGN)的病程。平均随访期为8年9个月;29名MPGN儿童(71%)通过学校尿液筛查确诊;32例为I型MPGN,2例为II型,7例为III型;10例患者接受多次低剂量联合疗法(MLD)治疗,8例先接受MLD治疗,随后接受大剂量隔日(ALD)泼尼松龙治疗,21例仅接受大剂量ALD泼尼松龙治疗。1例患者的MPGN进展为终末期肾衰竭。其余40例患者在最后一次随访时血清肌酐水平≤1.3mg/dl。24例(59%)患者的尿液分析正常。在仍有尿液异常的17例患者中,4例患有肾病综合征。接受大剂量ALD泼尼松龙治疗的患者尿液异常缓解率最高。对33例患者进行了重复肾活检,11例(33%)患者组织学有轻微改善,8例(24%)有中度改善,5例(15%)有显著改善,9例患者(27%)病情恶化或无改善。观察到的治疗副作用相对较少。与其他研究相比,本研究中MPGN患者的较好预后可能是早期检测和治疗的结果。

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