Suppr超能文献

激素敏感性肾病综合征治疗的最新进展

Update on the treatment of steroid-sensitive nephrotic syndrome.

作者信息

Zotta Federica, Vivarelli Marina, Emma Francesco

机构信息

Department of Pediatric Subspecialties, Division of Nephrology, Bambino Gesù Children's Hospital - IRCCS, Piazza S. Onofrio 4, 00165, Rome, Italy.

出版信息

Pediatr Nephrol. 2022 Feb;37(2):303-314. doi: 10.1007/s00467-021-04983-3. Epub 2021 Mar 5.

Abstract

Steroid-sensitive nephrotic syndrome (SSNS) is a rare condition that develops primarily in preadolescent children after the age of 1 year. Since the 1950s, oral corticosteroids have been the mainstay of treatment of all children presenting with nephrotic syndrome, with most patients responding within 4 weeks to an oral course of prednisone (PDN). However, corticosteroids have important side effects and 60-80 % of patients relapse, developing frequently relapsing or steroid-dependent forms. For these reasons, many patients require second-line steroid-sparing immunosuppressive medications that have considerably improved relapse-free survival, while avoiding many PDN-related toxicities. Since most patients will eventually heal from their disease with a normal kidney function, the morbidity of SSNS is primarily related to side effects of drugs that are used to maintain prolonged remission. Therefore, treatment is essentially based on balancing the use of different drugs to achieve permanent remission with the lowest cumulative number of side effects. Treatment choice is based on the severity of SSNS, on patient age, and on drug tolerability. This review provides an update of currently available therapeutic strategies for SSNS.

摘要

类固醇敏感性肾病综合征(SSNS)是一种罕见疾病,主要发生在1岁以后的青春期前儿童。自20世纪50年代以来,口服糖皮质激素一直是所有肾病综合征患儿的主要治疗方法,大多数患者在接受泼尼松(PDN)口服疗程后4周内有反应。然而,糖皮质激素有重要的副作用,60%-80%的患者会复发,常发展为频繁复发或类固醇依赖型。由于这些原因,许多患者需要二线类固醇节省免疫抑制药物,这些药物显著提高了无复发生存率,同时避免了许多与PDN相关的毒性。由于大多数患者最终会康复且肾功能正常,SSNS的发病率主要与用于维持长期缓解的药物的副作用有关。因此,治疗基本上基于平衡使用不同药物,以实现永久缓解并使副作用的累积数量降至最低。治疗选择基于SSNS的严重程度、患者年龄和药物耐受性。本综述提供了SSNS当前可用治疗策略的最新信息。

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验