As a good prognostic sign, Autosplenectomy is higher in females in Sickle Cell Diseases

Mehmet Rami Helvaci, Valeria Pappel, Kubra Piral, Selim Ozer, Mehpar Camlibel, Huseyin Sencan, Ramazan Davran, Abdulrazak Abyad, Lesley Pocock
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引用次数: 2

Abstract

Background: Sickle cell diseases (SCDs) are inborn and destructive processes on vascular endothelium, particularly at the capillaries. Methods: All patients with the SCDs were included. Results: We studied 222 males and 212 females with similar ages (30.8 vs 30.3 years, p>0.05, respectively). Although the higher prevalence of autosplenectomy (50.4% vs 53.3%, p<0.05), transfused units of red blood cells (RBCs) in their lives (48.1 vs 28.5, p=0.000), disseminated teeth losses (5.4% vs 1.4%, p<0.001), ileus (7.2% vs 1.4%, p<0.001), cirrhosis (8.1% vs 1.8%, p<0.001), leg ulcers (19.8% vs 7.0%, p<0.001), clubbing (14.8% vs 6.6%, p<0.001), coronary heart disease (CHD) (18.0% vs 13.2%, p<0.05), chronic renal disease (CRD) (9.9% vs 6.1%, p<0.05), chronic obstructive pulmonary disease (COPD) (25.2% vs 7.0%, p<0.001), and stroke (12.1% vs 7.5%, p<0.05) were all lower in females. Conclusion: The sickled or just hardened RBCs-induced capillary endothelial damage initiates at birth, and terminates with multiorgan failures even at childhood. Although RBCs suspensions and corticosteroids in acute, and aspirin with an anti-inflammatory dose plus low-dose warfarin plus hydroxyurea both in acute and chronic phases decrease severity, survivals are still shortened in both genders, dramatically. Although the higher prevalence of autosplenectomy, transfused units of RBCs in their lives disseminated teeth losses, ileus, cirrhosis, leg ulcers, clubbing, CHD, CRD, COPD, and stroke were all lower in females. So there may be an inverse relationship between autosplenectomy and severity of SCDs, and spleen may act as a chronic inflammatory focus as a filter of blood for these sickled or just hardened RBCs. Key words: Sickled or just hardened red blood cells, capillary endothelial edema, myocardial infarction, stroke, sudden deaths, autosplenectomy, female gender
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自体脾切除术是镰状细胞病患者的一个良好预后指标
背景:镰状细胞病(SCDs)是一种先天性的破坏血管内皮,尤其是毛细血管的疾病。方法:纳入所有SCDs患者。结果:我们研究了222名年龄相近的男性和212名女性(30.8 vs 30.3, p>0.05)。尽管自身脾切除术(50.4%比53.3%,p<0.05)、输注红细胞(红细胞)单位(48.1比28.5,p=0.000)、弥散性牙齿脱落(5.4%比1.4%,p<0.001)、肠梗阻(7.2%比1.4%,p<0.001)、肝硬化(8.1%比1.8%,p<0.001)、腿部溃疡(19.8%比7.0%,p<0.001)、棒状硬化(14.8%比6.6%,p<0.001)、冠心病(18.0%比13.2%,p<0.05)、慢性肾脏疾病(9.9%比6.1%,p<0.05)、慢性阻塞性肺疾病(COPD) (25.2% vs 7.0%, p < 0.001)和中风(12.1% vs 7.5%, p < 0.05)在女性中均较低。结论:红细胞诱导的镰状或刚硬化毛细血管内皮损伤始于出生,甚至在儿童时期就终止于多器官功能衰竭。尽管急性期红细胞混悬液和皮质类固醇,以及急性和慢性期抗炎剂量的阿司匹林加低剂量华法林加羟基脲均可降低严重程度,但男女患者的生存率仍显著缩短。尽管自体脾切除术的患病率较高,但她们生活中输血的红细胞单位弥散性牙齿缺失、肠梗阻、肝硬化、腿部溃疡、棒状硬化、冠心病、慢性阻塞性肺病和中风在女性中都较低。因此自体脾切除术和SCDs的严重程度之间可能存在反比关系,脾脏可能作为慢性炎症灶,作为镰状红细胞或硬化红细胞的血液过滤器。关键词:镰状或硬化红细胞,毛细血管内皮水肿,心肌梗死,中风,猝死,自体脾切除术,女性
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