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肾病综合征

Nephrotic syndrome.

作者信息

Andolino Tecile Prince, Reid-Adam Jessica

机构信息

St. Luke's University Health Network, Bethlehem, PA. Icahn School of Medicine at Mount Sinai, New York, NY. When this review was submitted, Dr. Andolino was in her third and final year of a pediatric nephrology fellowship at Sinai.

Icahn School of Medicine at Mount Sinai, New York, NY.

出版信息

Pediatr Rev. 2015 Mar;36(3):117-25; quiz 126, 129. doi: 10.1542/pir.36-3-117.

DOI:10.1542/pir.36-3-117
PMID:25733763
Abstract

On the basis of observational studies, the most common cause of nephrotic syndrome in school-aged children is minimal change disease. On the basis of research evidence and consensus, corticosteroids are considered first-line therapy for treatment of nephrotic syndrome. On the basis of consensus, prednisone therapy should be initiated at doses of 60 mg/m2 per day (2 mg/kg per day) administered for 4 to 6 weeks, followed by 40 mg/m2 per dose (1.5 mg/kg) every other day for at least 6 to 8 weeks. On the basis of consensus and expert opinion, it is important to recognize and manage the complications that can arise in patients with nephrotic syndrome, such as dyslipidemia, infection, and thrombosis. On the basis of research evidence, consensus, and expert opinion, several alternative therapies have been observed to have variable efficacy in children with both corticosteroid-dependent and corticosteroid-resistant nephrotic syndrome, although caution must be exercised in the administration of these corticosteroid-sparing medications secondary to toxic adverse effects. On the basis of observational studies, the course of nephrotic syndrome in most patients is that of relapse and remission.

摘要

基于观察性研究,学龄儿童肾病综合征最常见的病因是微小病变病。基于研究证据和共识,皮质类固醇被视为治疗肾病综合征的一线疗法。基于共识,泼尼松治疗应起始于每日60mg/m²(2mg/kg)的剂量,持续给药4至6周,随后隔日给予每剂40mg/m²(1.5mg/kg),至少持续6至8周。基于共识和专家意见,认识并处理肾病综合征患者可能出现的并发症,如血脂异常、感染和血栓形成,非常重要。基于研究证据、共识和专家意见,已观察到几种替代疗法在皮质类固醇依赖型和皮质类固醇抵抗型肾病综合征儿童中具有不同程度的疗效,不过由于这些皮质类固醇节省药物存在毒性不良反应,在给药时必须谨慎。基于观察性研究,大多数患者肾病综合征的病程为复发和缓解。

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Nephrotic syndrome.肾病综合征
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Management of nephrotic syndrome in childhood.儿童肾病综合征的管理
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[Multicenter study on present status of diagnosis and treatment of steroid-resistant nephrotic syndrome in children].[儿童激素抵抗型肾病综合征诊治现状的多中心研究]
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[Clinical application of tacrolimus with low-dose prednisone in 21 children with steroid-resistant nephrotic syndrome].他克莫司联合小剂量泼尼松在21例激素耐药型肾病综合征患儿中的临床应用
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