Elfaki Yousif Ahmed, Ibrahim Ahmed Saadeldin, Merghani Tarig Hakim
Department of Internal Medicine, Omdurman Teaching Hospital, Omdurman, Sudan.
Department of Medicine, Prince Sultan Military Medical City, Riyadh, KSA.
Open Respir Med J. 2024 May 21;18:e18743064292252. doi: 10.2174/0118743064292252240422100911. eCollection 2024.
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.
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.
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).
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.
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。SCD对肺血管系统的后续影响可导致肺动脉高压（PHT），这是一种严重的并发症，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地理区域和人群中存在差异。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。
招募了31名经血红蛋白电泳确诊的成年镰状细胞病（SCD）患者参与这项横断面研究。收集了包括人口统计学、临床和实验室参数在内的综合数据。采用多普勒超声心动图量化肺动脉收缩压（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%。主动吸烟与PHT有显著关联（P=0.042），而羟基脲治疗对PHT没有明显影响（P=0.612）。
我们的调查显示，在我们的SCD患者群体中，PHT患病率不到三分之一，与先前的研究一致。值得注意的是，独立于其他因素，吸烟是SCD患者发生PHT的一个明显危险因素。这突出了戒烟作为一种干预措施来延缓这种疾病发生的潜在效用。然而，迫切需要进一步研究以阐明吸烟导致SCD患者发生PHT的机制。
