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1
Hemostatic and thrombotic markers in patients with hemoglobin E/beta-thalassemia disease.血红蛋白E/β地中海贫血患者的止血和血栓形成标志物
Am J Hematol. 2007 Nov;82(11):1001-4. doi: 10.1002/ajh.20945.
2
Tissue plasminogen activator and plasminogen activator inhibitor type 1 gene polymorphism in patients with gastric ulcer complicated with bleeding.胃溃疡合并出血患者组织型纤溶酶原激活物及纤溶酶原激活物抑制剂-1基因多态性
J Korean Med Sci. 2003 Feb;18(1):58-64. doi: 10.3346/jkms.2003.18.1.58.
3
A chronic hypercoagulable state in patients with beta-thalassaemia major is already present in childhood.重型β地中海贫血患者的慢性高凝状态在儿童期就已存在。
Br J Haematol. 1999 Dec;107(4):739-46. doi: 10.1046/j.1365-2141.1999.01758.x.
4
A genetic polymorphism in connexin 37 as a prognostic marker for atherosclerotic plaque development.连接蛋白37基因多态性作为动脉粥样硬化斑块发展的预后标志物。
J Intern Med. 1999 Aug;246(2):211-8. doi: 10.1046/j.1365-2796.1999.00564.x.
5
Pulmonary hypertension in patients with thalassemia major.重型地中海贫血患者的肺动脉高压
Am Heart J. 1997 Sep;134(3):532-7. doi: 10.1016/s0002-8703(97)70091-7.
6
Thalassemia: lung function with reference to iron studies and reactive oxidant status.地中海贫血:与铁研究及活性氧状态相关的肺功能
Pediatr Hematol Oncol. 1993 Jan-Mar;10(1):13-23. doi: 10.3109/08880019309016523.
7
Cardiac dysfunction in beta-thalassemic children.β-地中海贫血患儿的心脏功能障碍
J Med Assoc Thai. 1993 Nov;76(11):591-6.
8
Allele-specific increase in basal transcription of the plasminogen-activator inhibitor 1 gene is associated with myocardial infarction.纤溶酶原激活物抑制剂1基因基础转录的等位基因特异性增加与心肌梗死相关。
Proc Natl Acad Sci U S A. 1995 Mar 14;92(6):1851-5. doi: 10.1073/pnas.92.6.1851.
9
Pulmonary artery obstruction in thalassaemia.地中海贫血中的肺动脉梗阻
Southeast Asian J Trop Med Public Health. 1980 Dec;11(4):516-23.
10
Right ventricular cardiac dysfunction in beta-thalassemia major.重型β地中海贫血中的右心室心脏功能障碍
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接受多次输血的重型地中海贫血患者的纤溶酶原激活物抑制剂-1研究

PAI-1 Study in Thalassemia Major Patients Receiving Multiple Blood Transfusion.

作者信息

Kumar Ashwini, Batra H S, Banerjee Mithu, Bandyopadhyay S, Saha T K, Misra Pratibha, Ambade Vivek

机构信息

Department of Biochemistry, Air Force Hospital, Jorhat, Assam 785005 India.

Department of Biochemistry, Armed Forces Medical College, Pune, Maharashtra 411040 India.

出版信息

Indian J Clin Biochem. 2017 Jul;32(3):343-346. doi: 10.1007/s12291-016-0620-7. Epub 2016 Oct 13.

DOI:10.1007/s12291-016-0620-7
PMID:28811695
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5539013/
Abstract

Thalassemia is a congenital hemolytic disease which is treated by repeated blood transfusion. Chronic iron overload is currently considered to be the primary cause of mortality in β-thalassemia, mainly due to the induction of left-sided cardiac failure. Iron overload results from a number of mechanisms associated with the disease itself. In addition to chronic iron overload thalassemic patients are more prone for procoagulant status which in turn lead to clinical thrombotic events. The hypercoagulable state in thalassemia is due to multiple elements, a combination of which is often the drive behind a clinical thromboembolic events. PAI-1 study was done in thalassemia major patients receiving multiple blood transfusion as a marker for procoagulant status. Total of 30 thalassemic patients on repeated blood transfusion was included in the study and total of 30 healthy age and sex matched controls were included in the study. It was also found that there was significant differences between cases and controls. The mean level of PAI 1 in controls was 3047 ± 414 pg/ml, the value in cases was 3683 ± 358 pg/ml. The level was significantly increased ( < 0.05) in the cases compared to controls. PAI-1 levels were also compared with the total number of blood transfusion which correlates well.

摘要

地中海贫血是一种先天性溶血性疾病,通过反复输血进行治疗。慢性铁过载目前被认为是β地中海贫血患者死亡的主要原因,主要是由于引发了左心衰竭。铁过载是由多种与疾病本身相关的机制导致的。除了慢性铁过载外,地中海贫血患者更容易出现促凝状态,进而导致临床血栓事件。地中海贫血的高凝状态是由多种因素引起的,这些因素的组合往往是临床血栓栓塞事件的背后驱动力。针对接受多次输血的重型地中海贫血患者进行了PAI - 1研究,以此作为促凝状态的标志物。该研究共纳入了30例接受反复输血的地中海贫血患者,以及30例年龄和性别匹配的健康对照者。研究还发现,病例组和对照组之间存在显著差异。对照组PAI - 1的平均水平为3047±414 pg/ml,病例组的值为3683±358 pg/ml。与对照组相比,病例组的水平显著升高(<0.05)。PAI - 1水平还与输血总数进行了比较,二者相关性良好。