M. Helvaci, Aysenur Varol, Kuddusi Onay, A. Abyad, Lesley Pocock
{"title":"低剂量华法林可能是一种挽救镰状细胞病生命的治疗方案","authors":"M. Helvaci, Aysenur Varol, Kuddusi Onay, A. Abyad, Lesley Pocock","doi":"10.5742/mewfm.2023.95256131","DOIUrl":null,"url":null,"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). 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 (18.0% vs 13.2%, p<0.05), chronic renal disease (9.9% vs 6.1%, p<0.05), chronic obstructive pulmonary disease (25.2% vs 7.0%, p<0.001), and stroke (12.1% vs 7.5%, p<0.05) were higher in males but not acute chest syndrome (2.7% vs 3.7%), pulmonary hypertension (12.6% vs 11.7), deep venous thrombosis and/or varices and/or telangiectasias (9.0% vs 6.6%), or mean age of mortality (30.2 vs 33.3 years) (p>0.05 for all). Conclusion: The sickled or just hardened red blood cells (RBCs)-induced capillary endothelial damage initiates at birth, and terminates with multiorgan failures and sudden deaths even at childhood. Although RBCs suspensions and corticosteroids in acute, and aspirin plus hydroxyurea both in acute and chronic phases decrease severity, survivals are still shortened in both genders, dramatically. Infections, medical or surgical emergencies, or emotional stress-induced increased basal metabolic rate accelerates sickling, and an exaggerated capillary endothelial edema-induced myocardial infarction or stroke may cause sudden deaths. Lifelong aspirin with an anti-inflammatory dose plus low-dose warfarin may be life-saving even at childhood both to decrease severity of capillary endothelial inflammation and to prevent thromboembolic complications in the SCDs. Key words: Sickle cell diseases, sickled or just hardened red blood cells, capillary endothelial edema, myocardial infarction, stroke, sudden deaths, low-dose warfarin","PeriodicalId":23895,"journal":{"name":"World Family Medicine Journal /Middle East Journal of Family Medicine","volume":"62 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"4","resultStr":"{\"title\":\"Low-dose warfarin may be a life-saving treatment regimen in sickle cell diseases\",\"authors\":\"M. Helvaci, Aysenur Varol, Kuddusi Onay, A. Abyad, Lesley Pocock\",\"doi\":\"10.5742/mewfm.2023.95256131\",\"DOIUrl\":null,\"url\":null,\"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). 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 (18.0% vs 13.2%, p<0.05), chronic renal disease (9.9% vs 6.1%, p<0.05), chronic obstructive pulmonary disease (25.2% vs 7.0%, p<0.001), and stroke (12.1% vs 7.5%, p<0.05) were higher in males but not acute chest syndrome (2.7% vs 3.7%), pulmonary hypertension (12.6% vs 11.7), deep venous thrombosis and/or varices and/or telangiectasias (9.0% vs 6.6%), or mean age of mortality (30.2 vs 33.3 years) (p>0.05 for all). Conclusion: The sickled or just hardened red blood cells (RBCs)-induced capillary endothelial damage initiates at birth, and terminates with multiorgan failures and sudden deaths even at childhood. Although RBCs suspensions and corticosteroids in acute, and aspirin plus hydroxyurea both in acute and chronic phases decrease severity, survivals are still shortened in both genders, dramatically. Infections, medical or surgical emergencies, or emotional stress-induced increased basal metabolic rate accelerates sickling, and an exaggerated capillary endothelial edema-induced myocardial infarction or stroke may cause sudden deaths. Lifelong aspirin with an anti-inflammatory dose plus low-dose warfarin may be life-saving even at childhood both to decrease severity of capillary endothelial inflammation and to prevent thromboembolic complications in the SCDs. 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引用次数: 4
摘要
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:男性222例,女性212例,年龄相近(30.8岁vs 30.3岁,p>0.05)。弥散性牙缺失(5.4% vs 1.4%, p < 0.05)。结论:镰状或刚硬化红细胞(rbc)诱导的毛细血管内皮损伤始于出生,并以多器官衰竭和儿童期猝死而终止。尽管红细胞悬液和皮质类固醇在急性期,阿司匹林加羟基脲在急性期和慢性期都能降低严重程度,但男女患者的生存时间仍显著缩短。感染、医疗或手术紧急情况或情绪压力引起的基础代谢率增加会加速镰状病变,而毛细血管内皮水肿引起的心肌梗死或中风可能导致猝死。终身服用抗炎剂量的阿司匹林加上低剂量的华法林,即使在儿童时期也可能挽救生命,既可以降低毛细血管内皮炎症的严重程度,又可以预防SCDs的血栓栓塞并发症。关键词:镰状细胞病,镰状或刚硬化的红细胞,毛细血管内皮水肿,心肌梗死,中风,猝死,低剂量华法林
Low-dose warfarin may be a life-saving treatment regimen in sickle cell diseases
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). 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 (18.0% vs 13.2%, p<0.05), chronic renal disease (9.9% vs 6.1%, p<0.05), chronic obstructive pulmonary disease (25.2% vs 7.0%, p<0.001), and stroke (12.1% vs 7.5%, p<0.05) were higher in males but not acute chest syndrome (2.7% vs 3.7%), pulmonary hypertension (12.6% vs 11.7), deep venous thrombosis and/or varices and/or telangiectasias (9.0% vs 6.6%), or mean age of mortality (30.2 vs 33.3 years) (p>0.05 for all). Conclusion: The sickled or just hardened red blood cells (RBCs)-induced capillary endothelial damage initiates at birth, and terminates with multiorgan failures and sudden deaths even at childhood. Although RBCs suspensions and corticosteroids in acute, and aspirin plus hydroxyurea both in acute and chronic phases decrease severity, survivals are still shortened in both genders, dramatically. Infections, medical or surgical emergencies, or emotional stress-induced increased basal metabolic rate accelerates sickling, and an exaggerated capillary endothelial edema-induced myocardial infarction or stroke may cause sudden deaths. Lifelong aspirin with an anti-inflammatory dose plus low-dose warfarin may be life-saving even at childhood both to decrease severity of capillary endothelial inflammation and to prevent thromboembolic complications in the SCDs. Key words: Sickle cell diseases, sickled or just hardened red blood cells, capillary endothelial edema, myocardial infarction, stroke, sudden deaths, low-dose warfarin