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非基因疗法在降低镰状细胞危象发生率中的作用:一项系统评价

The Role of Non-genetic Therapies to Reduce the Incidence of Sickle Cell Crisis: A Systematic Review.

作者信息

Pingili Shravya, Makkena Vijaya Krishna, Jaramillo Arturo P, Awosusi Babatope L, Ayyub Javaria, Dabhi Karan Nareshbhai, Gohil Namra V, Tanveer Nida, Hussein Sally, Hamid Pousette

机构信息

Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.

Pathology and Laboratory Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.

出版信息

Cureus. 2023 Aug 1;15(8):e42785. doi: 10.7759/cureus.42785. eCollection 2023 Aug.

Abstract

Sickle cell anemia is a hemoglobinopathy that causes complications such as Vaso-Occlusive Crisis (VOC), stroke, priapism, Acute Chest Syndromes (ACS), and bone infarcts due to blood vessel occlusion, resulting in hypoxia, ischemia, and inflammation. Preventing these incidents improves the quality of life and lowers mortality rates in Sickle Cell Disease (SCD) patients. This systematic review aims to describe the drugs, their mechanisms of action, dosages, changes in hemoglobin parameters, decrease in VOCs, delay the time for the next VOC, decrease in the length of hospital stay, and side effects associated with these drugs. This review adheres to the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) 2020 guidelines. For this review, we searched the PubMed, Google Scholar, and Cochrane databases and screened them for full free texts published in English and studied in humans in the last five years beginning in 2018. Randomized clinical trials (RCT), observational studies, meta-analyses, systemic reviews, and traditional reviews were all included in the search. According to the type of study, quality assessment tools are used, and eight papers are chosen. Full-text articles from these papers are studied, analyzed, and tabulated. We discussed seven interventions that are used to treat sickle cell disease. Voxelotor, crizanlizumab, L-glutamate, long-term blood transfusions, Zinc (Zn), Niprisan®, and Ciklavit* were found to reduce the number and severity of VOC. We discovered that VOCs containing L -glutamate reduced the length of hospitalization. Magnesium (Mg) did not affect the number and severity of VOCs. This review includes a few articles for the study. Future papers on this subject should include a large sample size and many papers. More clinical trials are required to evaluate the dosages and outcomes of using these drugs in combination to prevent VOCs.

摘要

镰状细胞贫血是一种血红蛋白病,会引发诸如血管闭塞性危机(VOC)、中风、阴茎异常勃起、急性胸综合征(ACS)以及因血管阻塞导致的骨梗死等并发症,进而造成缺氧、缺血和炎症。预防这些事件可改善镰状细胞病(SCD)患者的生活质量并降低死亡率。本系统评价旨在描述药物、其作用机制、剂量、血红蛋白参数变化、VOCs减少情况、下次VOC发生时间的延迟、住院时间缩短以及与这些药物相关的副作用。本评价遵循系统评价与Meta分析的首选报告项目(PRISMA)2020指南。对于本评价,我们检索了PubMed、谷歌学术和Cochrane数据库,并筛选了2018年开始的过去五年中以英文发表的、针对人类研究的全文。检索纳入了随机临床试验(RCT)、观察性研究、Meta分析、系统评价和传统综述。根据研究类型使用质量评估工具,并选取了八篇论文。对这些论文的全文进行研究、分析并制成表格。我们讨论了用于治疗镰状细胞病的七种干预措施。发现伏氯唑、crizanlizumab、L-谷氨酸、长期输血、锌(Zn)、Niprisan®和Ciklavit*可减少VOC的数量和严重程度。我们发现含L-谷氨酸的VOCs可缩短住院时间。镁(Mg)对VOC的数量和严重程度没有影响。本评价纳入了一些用于研究的文章。关于该主题的未来论文应包括大样本量且多篇论文。需要更多的临床试验来评估联合使用这些药物预防VOCs的剂量和效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be60/10469705/13f3abea5aa4/cureus-0015-00000042785-i01.jpg

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