Alzahrani Fatma, Fallatah Anas M, Al-Haddad Fatimah M, Khayyat Shahad T, AlMehmadi Wasayf M, AlQahtani Bashaier G, Alamri Rawabi S
Pediatrics, King Abdulaziz University Hospital, Jeddah, SAU.
Medicine, College of Medicine, King Abdulaziz University, Jeddah, SAU.
Cureus. 2021 Jan 3;13(1):e12440. doi: 10.7759/cureus.12440.
Background and objective Sickle cell anemia (SCA) is one of the common genetic diseases in the Kingdom of Saudi Arabia (KSA). This disease results from a genetic mutation that causes malformation of the red blood cells (RBCs), leading to various systemic complications, including vaso-occlusive crisis (VOC), acute chest syndrome (ACS), osteomyelitis, avascular necrosis (AVN), and stroke, to name a few. The leading cause of mortality in SCA is these systemic complications rather than the disease itself. Understanding the risk factors of these complications can help reduce mortality in these patients and improve their quality of life. In this study, we aimed to determine the risk factors of SCA complications among pediatric patients with SCA at King Abdulaziz University Hospital (KAUH) in Jeddah, KSA. Methods This retrospective study was carried out from January 2012 till June end 2019. It was conducted among pediatric patients with SCA. Patients were screened for eligibility, and we excluded those with thalassemia and those who had a medical history of chronic diseases. Data were collected from patients' electronic medical records. Results The study included 102 pediatric patients with SCA; their mean age was 7.88 ±4.22 years; almost half of them were females (56%) and 44% were males. The dominant body mass index (BMI) classification among them was normal (49%). Urinary tract infection (UTI) was the most common complication with 38 cases followed by VOC with 32 cases. Other complications observed were ACS (25.5%) followed by stroke (15.7%). HbSS was the most prominent genotype among these patients, and it was associated with a higher rate of complications. However, there was no significant relationship between genotype and patients developing complications. Finally, patients with high white blood cell (WBC) counts, elevated systolic blood pressure (SBP), and hypoxia developed more complications, and there was a significant relationship between these conditions and the development of complications (p<0.05). Conclusion Based on our findings, patients with high WBC count, elevated SBP, and hypoxia are at greater risk of developing complications. Accordingly, healthcare providers should consider putting in place all measures required to provide a good quality of life for these patients, including raising awareness about the risk factors that lead to these complications, appropriate immunizations, and precautionary measures to promote these patients' welfare.
背景与目的 镰状细胞贫血(SCA)是沙特阿拉伯王国(KSA)常见的遗传疾病之一。这种疾病由基因突变导致红细胞(RBC)畸形引起,会引发各种全身并发症,包括血管阻塞性危机(VOC)、急性胸部综合征(ACS)、骨髓炎、缺血性坏死(AVN)和中风等。SCA患者死亡的主要原因是这些全身并发症而非疾病本身。了解这些并发症的危险因素有助于降低这些患者的死亡率并改善其生活质量。在本研究中,我们旨在确定沙特阿拉伯吉达阿卜杜勒阿齐兹国王大学医院(KAUH)的小儿SCA患者中SCA并发症的危险因素。
方法 这项回顾性研究于2012年1月至2019年6月底进行。研究对象为小儿SCA患者。对患者进行资格筛查,排除患有地中海贫血和有慢性疾病病史的患者。数据从患者的电子病历中收集。
结果 该研究纳入了102例小儿SCA患者;他们的平均年龄为7.88±4.22岁;其中近一半为女性(56%),44%为男性。他们中占主导的体重指数(BMI)分类为正常(49%)。尿路感染(UTI)是最常见的并发症,有38例,其次是VOC,有32例。观察到的其他并发症依次为ACS(25.5%)和中风(15.7%)。HbSS是这些患者中最主要的基因型,且与较高的并发症发生率相关联。然而,基因型与患者发生并发症之间没有显著关系。最后,白细胞(WBC)计数高、收缩压(SBP)升高和缺氧的患者发生更多并发症,且这些情况与并发症的发生之间存在显著关系(p<0.05)。
结论 根据我们的研究结果,白细胞计数高、收缩压升高和缺氧的患者发生并发症的风险更大。因此,医疗保健提供者应考虑采取一切必要措施,为这些患者提供良好的生活质量,包括提高对导致这些并发症的危险因素的认识、进行适当的免疫接种以及采取预防措施以促进这些患者的健康。