UNASSIGNED: 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.
UNASSIGNED: 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.
■在2023年2月对MEDLINE进行的搜索(从一开始),Embase(从一开始),和LILACS/IBECS(1950年至2023年)数据库使用搜索词\“非典型溶血性尿毒综合征\”和\“拉丁美洲\”及其变体检索到51条记录(完整的论文和会议摘要)以英文出版,西班牙语,或者葡萄牙语。重复数据消除后,手动筛选标题/摘要和添加作者已知的文章,包括25篇文章,其中17篇(68%)是全文。所有文章均在2013年至2022年期间发表。文章包括队列研究,注册表分析,和来自阿根廷的病例报告,巴西,智利和哥伦比亚。总的来说,拉丁美洲患有aHUS的患者呈现出经典的流行病学特征,临床,以及与这种情况相关的遗传特征,如世界其他地区所述。根据报告的国家和时间,在拉丁美洲的HUS治疗主要与血浆疗法和/或eculizumab。据报道,eculizumab显著改善了几乎所有成人和儿科患者的aHUS相关结局.
■Eculizumab极大地改变了aHUS的自然病程,改善预后和患者预后。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。