关键词: Cigarette smoking Electrophoresis Hydroxyurea Pulmonary hypertension Sickle cell disease Sudanese

来  源:   DOI:10.2174/0118743064292252240422100911   PDF(Pubmed)

Abstract:
UNASSIGNED: Sickle Cell Disease (SCD) is a hereditary condition characterized by aberrant red blood cell morphology, leading to persistent hemolytic anemia. The consequential impact of SCD on the pulmonary vasculature can result in pulmonary hypertension (PHT), a severe complication that detrimentally affects the well-being and survival of individuals with SCD. The prevalence and risk determinants of PHT in SCD patients exhibit variations across diverse geographical regions and populations. This study aims to ascertain the prevalence of PHT among Sudanese SCD patients and identify associated factors.
UNASSIGNED: A cohort of thirty-one adult sickle cell disease (SCD) patients, as confirmed by hemoglobin electrophoresis, were recruited for participation in this cross-sectional study. Comprehensive data encompassing demographic, clinical, and laboratory parameters were collected. Doppler echocardiography was employed to quantify pulmonary arterial systolic pressure (PASP) and evaluate right ventricular size and function.
UNASSIGNED: Within our cohort, the prevalence of PHT was 29%. Active cigarette smoking demonstrated a significant association with PHT (P=0.042), while hydroxyurea therapy exhibited no noticeable impact on PHT (P=0.612).
UNASSIGNED: Our investigation revealed a PHT prevalence of less than one-third in our SCD patient population, aligning with prior studies. Notably, independent of other factors, cigarette smoking emerged as a distinct risk factor for PHT in SCD patients. This highlights the potential utility of smoking cessation as an intervention to delay the onset of this condition. However, further research is imperative to elucidate the mechanisms through which smoking contributes to PHT development in individuals with SCD.
摘要:
镰状细胞病(SCD)是一种遗传性疾病,其特征是红细胞形态异常,导致持续性溶血性贫血。因此,SCD对肺血管系统的影响可导致肺动脉高压(PHT),严重的并发症,对SCD患者的健康和生存产生不利影响。SCD患者中PHT的患病率和风险决定因素在不同的地理区域和人群中表现出差异。本研究旨在确定苏丹SCD患者中PHT的患病率并确定相关因素。
一组31名成人镰状细胞病(SCD)患者,正如血红蛋白电泳所证实的,被招募参加这项横断面研究。包括人口统计在内的全面数据,临床,并收集实验室参数。多普勒超声心动图用于量化肺动脉收缩压(PASP)并评估右心室大小和功能。
在我们的队列中,PHT的患病率为29%。积极吸烟与PHT显著相关(P=0.042)。而羟基脲治疗对PHT无明显影响(P=0.612)。
我们的调查显示,在我们的SCD患者人群中,PHT患病率不到三分之一,与先前的研究保持一致。值得注意的是,独立于其他因素,吸烟是SCD患者发生PHT的明显危险因素.这凸显了戒烟作为延缓这种情况发作的干预措施的潜在效用。然而,进一步的研究对于阐明吸烟促进SCD患者PHT发展的机制至关重要.
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