背景:镰状细胞病(SCD)是一种遗传性红细胞疾病,其中突变导致谷氨酸在β-珠蛋白链的第六个位置取代为缬氨酸。这些包括镰状细胞性贫血(纯合镰状突变),镰状β地中海贫血,和血红蛋白SCD。SCD的临床表现是变形蛋白。患有SCD的人患有急性和慢性并发症,其中包括经常发作的疼痛,通常称为血管闭塞性危机(VOC)-急性胸部综合征(ACS);骨无菌性坏死;脾脏微梗塞,大脑,和肾脏;感染;中风;以及影响身体各个部位的器官损伤。由于严重的并发症,SCD需要频繁住院,这给护理人员带来了巨大的负担,给医疗保健系统带来了经济压力。SCD的入院模式在世界不同地区有所不同。
目的:本研究旨在确定青少年和成人SCD患者住院的原因,并确定与住院时间相关的因素。
方法:该研究是一项以医院为基础的前瞻性观察性研究,包括诊断为SCD的青少年和成人患者,年龄在15-45岁之间,他们于2021年8月至2022年8月在莱普尔的全印度医学科学研究所的普通医学系住院。
结果:根据我们的研究,住院的主要原因是痛苦的危机,占63%的病例,其次是感染(17%),ACS(11%),和急性溶血性危象(9%)。值得注意的是,我们没有观察到性别和入院原因之间的任何显着差异(p>0.05)。关节痛(p=0.005),背痛(p=0.001),19岁以上的成年人报告胸痛(p=0.001)的发生率更高.此外,我们对住院时间和各种因素的分析显示,因感染而入院的患者平均住院时间明显更长(p=0.040).
结论:急性疼痛危象是SCD患者入院的主要原因;许多患者还遇到感染和ACS。此外,发生感染和VOC的患者的住院时间更长.因此,必须向他们提供有关针对感染的各种预防措施和引发痛苦危机的因素的全面指导。
BACKGROUND: Sickle cell disease (SCD) is an inherited red blood cell disorder, wherein mutation causes the substitution of glutamic acid to valine at the sixth position of the β-globin chain. These include sickle cell anemia (homozygous sickle mutation), sickle-beta thalassemia, and hemoglobin SCD. The clinical manifestations of SCD are protean. Individuals with SCD suffer from both acute and chronic complications, which include recurring episodes of pain commonly called vaso-occlusive crisis (VOC) - acute chest syndrome (ACS); aseptic necrosis of the bone; micro-infarction of the spleen, brain, and kidney; infections; stroke; and organ damage affecting every part of the body. SCD necessitates frequent hospitalizations because of severe complications, which pose a significant burden on caregivers and economic strain on healthcare systems. The pattern of hospital admission with SCD varies in different parts of the world.
OBJECTIVE: This study aimed to determine the causes of hospitalization among adolescent and adult patients with SCD and to determine factors associated with their hospital stay.
METHODS: The study was a hospital-based prospective observational study comprising adolescent and adult patients diagnosed with SCD, aged 15-45 years, who were hospitalized in the Department of General Medicine at All India Institute of Medical Sciences in Raipur from August 2021 to August 2022.
RESULTS: According to our study, the primary reason for hospitalization was a painful crisis, accounting for 63% of cases, followed by infection (17%), ACS (11%), and acute hemolytic crisis (9%). Notably, we did not observe any significant differences between genders and causes of admission (p > 0.05). Joint pain (p = 0.005), back pain (p = 0.001), and chest pain (p = 0.001) were more frequently reported by adults over the age of 19. In addition, our analysis of the duration of hospital stays and various factors revealed that patients admitted for infections had a significantly longer mean hospital stay duration (p = 0.040).
CONCLUSIONS: Acute painful crises were the primary cause of hospital admission among individuals with SCD; many patients also encountered infections and ACS. Furthermore, patients who experienced infections and VOC had a lengthier duration of hospital stay. Therefore, it is essential to provide them with comprehensive instructions on various preventive measures against infections and the factors that trigger painful crises.