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儿童类固醇敏感性肾病综合征:复发诱因及发病机制的演变假说

Steroid-sensitive nephrotic syndrome in children: triggers of relapse and evolving hypotheses on pathogenesis.

作者信息

Uwaezuoke Samuel N

机构信息

Department of Paediatric Nephrology Unit, University of Nigeria Teaching Hospital, Postal code- 400001, Ituku-Ozalla, Enugu, Enugu State, Nigeria.

出版信息

Ital J Pediatr. 2015 Mar 21;41:19. doi: 10.1186/s13052-015-0123-9.

Abstract

Nephrotic syndrome remains the most common manifestation of glomerular disease in childhood. Minimal change nephropathy is the most common cause of the syndrome in children. Despite its initial high response rate to corticosteroids and its favorable prognosis, relapses are common leading to increased morbidity and cost of treatment.This review seeks to appraise the common triggers of relapse and to highlight the evolving hypotheses about the pathogenesis of the syndrome. Literature search was conducted through PubMed, Google web search and Cochrane Database of Systematic reviews using relevant search terms.Acute respiratory infections and urinary tract infections are the most frequent infectious triggers of relapse. Targeted interventions like initiating corticosteroid or its dose-adjustment during episodes of acute respiratory infection and zinc supplementation are reportedly effective in reducing relapse rates. Hypotheses on pathogenesis of the syndrome have evolved from the concepts of 'immune dysregulation', 'increased glomerular permeability' to 'podocytopathy'.Although development of drugs which can regulate the pathways for podocyte injury offers future hope for effective and targeted treatment, the relapse-specific interventions currently contribute to significant reduction in disease morbidity.

摘要

肾病综合征仍然是儿童肾小球疾病最常见的表现形式。微小病变肾病是儿童该综合征最常见的病因。尽管其最初对皮质类固醇的反应率较高且预后良好,但复发很常见,导致发病率增加和治疗成本上升。本综述旨在评估复发的常见触发因素,并强调关于该综合征发病机制的不断演变的假说。通过使用相关搜索词在PubMed、谷歌网络搜索和Cochrane系统评价数据库中进行文献检索。急性呼吸道感染和尿路感染是复发最常见的感染性触发因素。据报道,在急性呼吸道感染发作期间启动皮质类固醇或调整其剂量以及补充锌等针对性干预措施可有效降低复发率。关于该综合征发病机制的假说已从“免疫失调”“肾小球通透性增加”的概念演变为“足细胞病”。尽管能够调节足细胞损伤途径的药物的开发为有效和靶向治疗带来了未来希望,但目前针对复发的干预措施显著降低了疾病发病率。

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