A comprehensive review of diverse issues related to sickle cell disease.

H S Nikhar, S U Meshram, G B Shinde
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Abstract

Objective: This review was carried out to understand diverse issues in sickle cell disease (SCD), such as its prevalence, clinical manifestations, preventive measures, secondary infections, nutrition and therapies.

Methods: A deductive reasoning approach was followed to unearth the information from the published literature. Vast amount of resources (online and offline) were referred to collect and analyse the content related to SCD. The research publications were analysed following the standard content analysis procedures.

Results: The study results revealed a dearth of literature. The available information seems inconclusive due to the local nature of the disease, the high prevalence in the developing world where the accessibility to healthcare is a big issue. The prevalence data is scarce; the studies conducted so far reveal a very small sample size making the generalization of results difficult.

Conclusion: The review of literature for diverse issues related to Sickle cell disease (SCD) revealed varied aspects to address the SCD related issues. Although there are reports that provide interesting insights of SCD, majority of them are inconclusive. The prevalence reported by different authors varies significantly that warrants initiation of a comprehensive SCD screening programme. The pathophysiological changes in the patients with SCD present a challenging and dynamic situation, which needs to be studied in more details. The many clinical complications reported by different authors call for a more concerted effort for additional understanding. Furthermore, the studies conducted so far are found to have very less sample size, especially in light of the prevalence estimates reported by various authors. The review indicates the important aspects of SCD that are to be addressed in urgency such as follow-up of newborns screening tests, stroke prevention, pregnancy complications, pain prevention, morbidity and mortality studies, and genetic studies for the better life of people with SCD.

与镰状细胞病有关的各种问题的全面审查。
目的:本综述旨在了解镰状细胞病(SCD)的患病率、临床表现、预防措施、继发感染、营养和治疗等多种问题。方法:采用演绎推理的方法,从已发表的文献中挖掘信息。大量的资源(线上和线下)被用于收集和分析与SCD相关的内容。按照标准内容分析程序对研究出版物进行分析。结果:研究结果显示文献匮乏。现有的信息似乎不确定,因为这种疾病是地方性的,在发展中国家发病率很高,获得医疗保健是一个大问题。患病率数据很少;迄今为止进行的研究显示样本量非常小,使得结果难以推广。结论:回顾与镰状细胞病(SCD)相关的各种问题的文献,揭示了解决SCD相关问题的不同方面。虽然有一些报告提供了关于SCD的有趣见解,但大多数都是不确定的。不同作者报告的患病率差异很大,值得开展全面的SCD筛查计划。SCD患者的病理生理变化具有挑战性和动态性,需要进一步深入研究。不同作者报告的许多临床并发症需要更加协调一致的努力来进一步了解。此外,迄今为止进行的研究发现样本量非常少,特别是考虑到不同作者报告的流行率估计数。该综述指出了SCD急需解决的重要方面,如新生儿筛查试验的随访、中风预防、妊娠并发症、疼痛预防、发病率和死亡率研究以及SCD患者改善生活的基因研究。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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