ATHEROSCLEROTIC BACKGROUND OF DIGITAL CLUBBING IN SICKLE CELL DISEASES

M. Helvaci, Valeria Pappel, Kubra Piral, Asuman Caylar, Huseyin Sencan, R. Davran, Mustafa Yaprak, A. Abyad, Lesley Pocock
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Abstract

Background: Sickle cell diseases (SCDs) are inborn and catastrophic processes on vascular endothelium, particularly at the capillaries. Methods: All patients were included. Results: We studied 222 males and 212 females with similar mean ages (30.8 vs 30.3 years, p>0.05, respectively). Beside digital clubbing (14.8% vs 6.6%, p<0.001), 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), 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, significantly (30.2 vs 33.3 years, p<0.05). Conclusion: The hardened RBCs-induced capillary endothelial damage initiates at birth, and terminates with multiorgan failures even at childhood. Parallel to digital clubbing, all of the atherosclerotic risk factors or consequences including smoking, alcohol, disseminated teeth losses, COPD, ileus, cirrhosis, leg ulcers, CHD, CRD, and stroke were 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 the relatively younger mean age. So autosplenectomy may be a good whereas male gender alone may be a bad prognostic factor, and digital clubbing may have an atherosclerotic background in SCDs. Key words: Sickle cell diseases, hardened red blood cells, capillary endothelial damage, capillary endothelial edema, sudden deaths, atherosclerosis, digital clubbing
镰状细胞病中数字clubbing的动脉粥样硬化背景
背景:镰状细胞病(SCDs)是对血管内皮,尤其是毛细血管造成灾难性影响的先天性疾病。研究方法纳入所有患者。结果我们研究了平均年龄相似的 222 名男性和 212 名女性(分别为 30.8 岁和 30.3 岁,P>0.05)。除了数字性跛行(14.8% 对 6.6%,P<0.001)、吸烟(23.8% 对 6.1%,P<0.001)、酗酒(4.9% 对 0.4%,P<0.001)、生前输过红细胞(48.1 对 28.5 单位,P=0.000)、播散性牙齿脱落(5.4% vs 1.4%,P<0.001)、慢性阻塞性肺病(COPD)(25.2% vs 7.0%,P<0.001)、回肠炎(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)、冠心病(CHD)(18.0% vs 13.2%,p<0.05)、慢性肾病(CRD)(9.9% vs 6.1%,p<0.05)和中风(12.1% vs 7.5%,P<0.05)均较高,而男性的自体脾切除率(50.4% vs 53.3%,P<0.05)和平均死亡年龄明显较低(30.2 vs 33.3 岁,P<0.05)。结论红细胞硬化引起的毛细血管内皮损伤始于出生时,甚至在儿童期就以多器官功能衰竭而告终。与数字俱乐部同时出现的所有动脉粥样硬化危险因素或后果,包括吸烟、酗酒、牙齿脱落、慢性阻塞性肺病、回肠炎、肝硬化、腿部溃疡、冠心病、慢性阻塞性肺病和中风,在男性中都较高,而在平均年龄相对较小的男性中,自体脾切除术和平均死亡年龄都较低,这不能仅用吸烟和酗酒的影响来解释。因此,自体脾切除可能是一个好的预后因素,而单纯的男性性别可能是一个坏的预后因素,SCDs中的数字clubbing可能有动脉粥样硬化的背景。关键词镰状细胞病、硬化的红细胞、毛细血管内皮损伤、毛细血管内皮水肿、猝死、动脉粥样硬化、数字俱乐部
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