男性性别本身可能是镰状细胞病的不良预后因素

Mehmet Rami Helvaci, Valeria Pappel, Kubra Piral, Selim Ozer, Mehpare Camlibel, Huseyin Sencan, Ramazan Davran, Abdulrazak Abyad, Lesley Pocock
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摘要

背景:镰状细胞病是一种先天性的破坏血管内皮的疾病,尤其是毛细血管。方法:纳入所有患者。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)。吸烟(23.8% vs 6.1%, p<0.001)、饮酒(4.9% vs 0.4%, p<0.001)、一生中输注的红细胞(红细胞)(48.1 vs 28.5单位,p=0.000)、弥散性牙齿脱落(5.4% vs 1.4%, p<0.001)、慢性阻塞性肺疾病(COPD) (25.2% vs 7.0%, p<0.001)、肠道梗阻(7.2% vs 1.4%, p<0.001)、肝硬化(8.1% vs 1.8%, p<0.001)、腿部溃疡(19.8% vs 7.0%, p<0.001)、殴打(14.8% vs 6.6%, p<0.001)、冠心病(18.0% vs 13.2%, p<0.05)、慢性肾脏疾病(CRD) (9.9% vs 6.1%, p<0.05)和中风(12.1% vs 7.5%, p<0.05)均较高,男性自体脾切除术(50.4% vs 53.3%, p<0.05)和平均死亡率年龄较低(30.2 vs 33.3岁,p<0.05)。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。他们一生中输血的红细胞单位、弥散性牙齿脱落、COPD、肠阻、肝硬化、腿部溃疡、棒状硬化、冠心病、CRD和中风都较高,男性的自体脾切除术和平均死亡率较低,这不能单独用吸烟和酒精的影响来解释相对较年轻的平均年龄。关键词:镰状细胞病,镰状或刚硬化红细胞,毛细血管内皮水肿,心肌梗死,中风,猝死,男性
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Male Gender alone may be a bad Prognostic Factor in Sickle Cell Diseases
Background: Sickle cell diseases are inborn and destructive processes on vascular endothelium, particularly at the capillaries. Methods: All patients were included. Results: We studied 222 males and 212 females with similar ages (30.8 vs 30.3 years, p>0.05, respectively). Smoking (23.8% vs 6.1%, p<0.001), alcohol (4.9% vs 0.4%, p<0.001), transfused red blood cells (RBCs) in their lives (48.1 vs 28.5 units, p=0.000), disseminated teeth losses (5.4% vs 1.4%, p<0.001), chronic obstructive pulmonary disease (COPD) (25.2% vs 7.0%, 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), and stroke (12.1% vs 7.5%, p<0.05) were all higher, and autosplenectomy (50.4% vs 53.3%, p<0.05) and mean age of mortality were lower in males (30.2 vs 33.3 years, p<0.05). 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. Transfused units of RBCs in their lives, disseminated teeth losses, COPD, ileus, cirrhosis, leg ulcers, clubbing, CHD, CRD, and stroke were all higher, and autosplenectomy and mean age of mortality were lower in males which can not be explained by effects of smoking and alcohol alone at these younger mean ages, relatively. Key words: Sickle cell diseases, sickled or just hardened red blood cells, capillary endothelial edema, myocardial infarction, stroke, sudden deaths, male gender
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