拉丁美洲非典型溶血性尿毒症综合征的临床表现和治疗:文献综述。

IF 2.3 4区 医学 Q2 HEMATOLOGY Expert Review of Hematology Pub Date : 2024-07-01 Epub Date: 2024-06-12 DOI:10.1080/17474086.2024.2365169
R A Sepúlveda Palamara, L G Modelli de Andrade, R M Fortunato, B Gómez, J F Nieto-Ríos
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引用次数: 0

摘要

导言:欧洲和北美以外地区有关非典型溶血性尿毒症综合征(aHUS)的综合信息相对较少。这篇叙述性综述汇集了有关拉丁美洲非典型溶血性尿毒症的临床表现和管理的已发表数据:2023 年 2 月,我们使用 "非典型溶血性尿毒症综合征 "和 "拉丁美洲 "及其变体作为检索词,对 MEDLINE(从开始时起)、Embase(从开始时起)和 LILACS/IBECS (1950 年至 2023 年)数据库进行了检索,共检索到 51 条以英语、西班牙语或葡萄牙语发表的记录(论文全文和会议摘要)。经过去重、人工筛选标题/摘要和添加作者已知的文章后,共收录了 25 篇文章,其中 17 篇(68%)为论文全文。所有文章均发表于 2013-2022 年间。文章包括来自阿根廷、巴西、智利和哥伦比亚的队列研究、登记分析和病例报告。总体而言,拉丁美洲的 aHUS 患者具有世界其他地区所描述的典型流行病学、临床和遗传特征。根据国家和报告时间的不同,拉丁美洲的 aHUS 主要采用血浆疗法和/或依库珠单抗进行治疗。在有报道的地方,几乎所有成人和儿童患者的 aHUS 相关预后都得到了依库珠单抗的大幅改善:专家观点:依库珠单抗极大地改变了aHUS的自然病程,改善了预后和患者预后。应对经济挑战和投资医疗基础设施对于在拉丁美洲实施及时发现和早期治疗 aHUS 的战略至关重要。
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Clinical presentation and management of atypical hemolytic uremic syndrome in Latin America: a narrative review of the literature.

Introduction: Comprehensive information about atypical hemolytic uremic syndrome (aHUS) is relatively scarce outside of Europe and North America. This narrative review assembles available published data about the clinical presentation and management of aHUS in Latin America.

Areas covered: A search conducted in February 2023 of the MEDLINE (from inception), Embase (from inception), and LILACS/IBECS (1950 to 2023) databases using search terms 'atypical hemolytic uremic syndrome' and 'Latin America' and their variations retrieved 51 records (full papers and conference abstracts) published in English, Spanish, or Portuguese. After de-duplication, manual screening of titles/abstracts and addition of author-known articles, 25 articles were included of which 17 (68%) are full papers. All articles were published during the years 2013-2022. Articles include cohort studies, a registry analysis, and case reports from Argentina, Brazil, Chile and Columbia. Overall, Latin American patients with aHUS present the classic epidemiological, clinical, and genetic characteristics associated with this condition as described in other world regions. Depending on the country and time of reporting, aHUS in Latin America was treated mainly with plasma therapy and/or eculizumab. Where reported, eculizumab substantially improved aHUS-related outcomes in almost all adult and pediatric patients.

Expert opinion: Eculizumab has dramatically altered the natural course of aHUS, improving prognosis and patient outcomes. Addressing economic challenges and investing in healthcare infrastructure will be essential to implement strategies for timely detection and early treatment of aHUS in Latin America.

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来源期刊
CiteScore
4.70
自引率
3.60%
发文量
98
审稿时长
6-12 weeks
期刊介绍: Advanced molecular research techniques have transformed hematology in recent years. With improved understanding of hematologic diseases, we now have the opportunity to research and evaluate new biological therapies, new drugs and drug combinations, new treatment schedules and novel approaches including stem cell transplantation. We can also expect proteomics, molecular genetics and biomarker research to facilitate new diagnostic approaches and the identification of appropriate therapies. Further advances in our knowledge regarding the formation and function of blood cells and blood-forming tissues should ensue, and it will be a major challenge for hematologists to adopt these new paradigms and develop integrated strategies to define the best possible patient care. Expert Review of Hematology (1747-4086) puts these advances in context and explores how they will translate directly into clinical practice.
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