Department of Infectious-Tropical Diseases and Microbiology, IRCCS Sacro Cuore Don Calabria Hospital, Negrar di Valpolicella (Verona), Italy.
Department of Infectious-Tropical Diseases and Microbiology, IRCCS Sacro Cuore Don Calabria Hospital, Negrar di Valpolicella (Verona), Italy.
Clin Microbiol Infect. 2024 Jan;30(1):59-65. doi: 10.1016/j.cmi.2023.09.009. Epub 2023 Sep 20.
Blackwater fever (BWF) is a severe syndrome occurring in patients with malaria upon antimalarial treatment, characterized by massive intravascular haemolysis and haemoglobinuria. BWF is a neglected condition and management recommendations are unavailable.
We performed a scoping review to appraise available data on clinical picture, treatment and physiopathology of BWF, which could guide rationally its clinical management.
MEDLINE, EMBASE, LILACS, Web of Science, and Scopus databases, and the reference list of relevant publications, were searched. Papers reporting original data on BWF cases or investigating the physiopathology of BWF were eligible. Data regarding case characteristics, trigger event, clinical management and outcome were extracted. For papers investigating the physiopathology of BWF, study design and principal findings were extracted. No quality assessment was performed. Data are presented as numbers and percentages, and summary of findings, grouped by paper focus (clinical description or physiopathology).
101 papers were included. The majority of BWF cases were observed in autochthonous children (75.7%) and adults (15.3%), in contrast with historical perception that BWF patients were typically expatriates. Clinical management was described for 794 cases; corticosteroids were used in 23. Outcome was reported for 535 patients, with 18.1% mortality. The trigger was reported for 552 (47.5%) cases; in 70.4% identified as quinine. However, two RCT comparing artesunate and quinine for falciparum malaria treatment did not find significant difference in BWF occurrence after their administration. Two case-control studies did not find significant difference in G6PDH deficiency between malaria patients with and without BWF.
The physiopathology and optimal treatment of BWF remain similarly unknown as they were over a century ago. Empirical supporting treatment approach seems reasonable, while change of antimalarial drug and use of corticosteroids remain object of debate.
黑水热（BWF）是疟疾患者在接受抗疟治疗时发生的一种严重综合征，其特征为大量血管内溶血和血红蛋白尿。BWF 是一种被忽视的疾病，目前尚无管理建议。
我们进行了范围界定审查，以评估关于 BWF 的临床特征、治疗和病理生理学的现有数据，这些数据可指导其临床管理。
检索 MEDLINE、EMBASE、LILACS、Web of Science 和 Scopus 数据库以及相关文献的参考文献列表。报告 BWF 病例或调查 BWF 病理生理学的原始数据的论文符合纳入标准。提取病例特征、触发事件、临床管理和结局的数据。对于调查 BWF 病理生理学的论文，提取研究设计和主要发现。未进行质量评估。数据以数字和百分比表示，并根据论文重点（临床描述或病理生理学）进行汇总。
共纳入 101 篇论文。大多数 BWF 病例发生在土生土长的儿童（75.7%）和成年人（15.3%）中，与历史上认为 BWF 患者通常为外来移民的看法形成对比。描述了 794 例 BWF 病例的临床管理；23 例使用了皮质类固醇。535 例患者报告了结局，死亡率为 18.1%。552 例（47.5%）病例报告了触发因素；其中 70.4%的病例被确认为奎宁。然而，两项比较青蒿琥酯和奎宁治疗恶性疟的 RCT 发现，在接受治疗后，BWF 的发生没有显著差异。两项病例对照研究未发现 BWF 疟疾患者与非 BWF 疟疾患者之间的 G6PDH 缺乏有显著差异。
BWF 的病理生理学和最佳治疗方法与一个多世纪前一样，仍然知之甚少。经验性支持治疗方法似乎合理，而抗疟药物的改变和皮质类固醇的使用仍然存在争议。
