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揭示镰状细胞病血管闭塞性危象的复杂性:来自资源有限环境的见解

Unraveling the Complexity of Vaso-Occlusive Crises in Sickle Cell Disease: Insights from a Resource-Limited Setting.

作者信息

Kaponda Ali, Muya Kalunga, Panda Jules, Koto Kodondi Kule, Bonnechère Bruno

机构信息

Reference Centre for Sickle Cell Disease of Lubumbashi, Institut de Recherche en Science de la Santé, Lubumbashi 1825, Democratic Republic of the Congo.

Department of Clinical Biology, Faculty of Pharmaceutical Sciences, University of Lubumbashi, Lubumbashi 1825, Democratic Republic of the Congo.

出版信息

J Clin Med. 2024 Apr 25;13(9):2528. doi: 10.3390/jcm13092528.

Abstract

: This study investigated vaso-occlusive crises (VOCs) in sickle cell disease in Lubumbashi, Democratic Republic of Congo, aiming to understand the disease complexities amidst limited resources. With sickle cell hemoglobinopathies on the rise in sub-Saharan Africa, this nine-year study explored factors associated with VOCs and hematological components. : This study comprised 838 patients, analyzing VOCs and hematological changes over time. Demographic characteristics and blood composition changes were carefully categorized. A total of 2910 crises were observed and managed, with analyses conducted on severity, localization, and age groups using statistical methods. : The majority of crises were mild or moderate, primarily affecting osteoarticular regions. Statistical analysis revealed significant disparities in crisis intensity based on location and age. The association between blood samples and the number of comorbidities was investigated. Significant positive associations were found for all parameters, except monocytes, indicating a potential link between blood variables and complication burden. Survival analysis using Cox regression was performed to predict the probability of experiencing a second crisis. No significant effects of medication or localization were observed. However, intensity ( < 0.001), age ( < 0.001), and gender ( < 0.001) showed significant effects. Adjusted Hazard Ratios indicated increased risk with age and male gender and reduced risk with mild or severe crisis intensity compared to light. : This research sheds light on the complexities of VOCs in resource-limited settings where sickle cell disease is prevalent. The intricate interplay between clinical, laboratory, and treatment factors is highlighted, offering insights for improved patient care. It aims to raise awareness of patient challenges and provide valuable information for targeted interventions to alleviate their burden.

摘要

本研究调查了刚果民主共和国卢本巴希镰状细胞病的血管闭塞性危机(VOCs),旨在了解在资源有限的情况下该疾病的复杂性。随着撒哈拉以南非洲镰状细胞血红蛋白病的发病率上升,这项为期九年的研究探索了与VOCs和血液学成分相关的因素。本研究纳入了838名患者,分析了VOCs和血液学随时间的变化。仔细分类了人口统计学特征和血液成分变化。共观察和处理了2910次危机,并使用统计方法对严重程度、发生部位和年龄组进行了分析。大多数危机为轻度或中度,主要影响骨关节区域。统计分析显示,危机强度在发生部位和年龄方面存在显著差异。研究了血样与合并症数量之间的关联。除单核细胞外,所有参数均发现显著正相关,表明血液变量与并发症负担之间可能存在联系。使用Cox回归进行生存分析以预测再次发生危机的概率。未观察到药物或发生部位的显著影响。然而,强度(<0.001)、年龄(<0.001)和性别(<0.001)显示出显著影响。调整后的风险比表明,与轻度危机相比,年龄和男性性别会增加风险,而轻度或重度危机强度会降低风险。这项研究揭示了在镰状细胞病流行的资源有限环境中VOCs的复杂性。突出了临床、实验室和治疗因素之间复杂的相互作用,为改善患者护理提供了见解。其目的是提高对患者面临挑战的认识,并为有针对性的干预措施提供有价值的信息,以减轻他们的负担。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed0d/11084179/cb388bf4c46b/jcm-13-02528-g001.jpg

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