由于α2-抗纤溶酶缺乏引起的出血性疾病。
A bleeding disorder due to deficiency of alpha 2-antiplasmin.
作者信息
Kettle P, Mayne E E
出版信息
J Clin Pathol. 1985 Apr;38(4):428-9. doi: 10.1136/jcp.38.4.428.
Abstract
The investigation of a 33 year old man with a lifelong bleeding tendency is described. Defective fibrinolysis was suspected in 1968, when clinical bleeding was corrected by administration of aminocaproic acid. The paper establishes the diagnosis as alpha 2-antiplasmin deficiency and describes its management with oral tranexamic acid.
摘要
本文描述了一名有终生出血倾向的33岁男性的调查情况。1968年怀疑存在纤维蛋白溶解功能缺陷，当时通过给予氨基己酸纠正了临床出血。本文确诊为α2-抗纤溶酶缺乏症，并描述了用口服氨甲环酸进行治疗的情况。
相似文献
1
A bleeding disorder due to deficiency of alpha 2-antiplasmin.由于α2-抗纤溶酶缺乏引起的出血性疾病。
J Clin Pathol. 1985 Apr;38(4):428-9. doi: 10.1136/jcp.38.4.428.
2
Plasma alpha 2-antiplasmin activity. Role in the evaluation and management of fibrinolytic states and other bleeding disorders.血浆α2-抗纤溶酶活性。在纤溶状态及其他出血性疾病评估和管理中的作用。
Arch Intern Med. 1989 Aug;149(8):1769-72. doi: 10.1001/archinte.149.8.1769.
3
Inherited disorders of the fibrinolytic pathway.纤维蛋白溶解途径的遗传性疾病。
Transfus Apher Sci. 2019 Oct;58(5):572-577. doi: 10.1016/j.transci.2019.08.007. Epub 2019 Aug 8.
4
[Congenital deficiency of the fibrinolysis inhibitor alpha 2-antiplasmin as a cause of hemorrhagic diathesis].
Ned Tijdschr Geneeskd. 1988 Jan 23;132(4):167-70.
5
Epsilon-aminocaproic acid in the treatment of patients with acute promyelocytic leukemia and acquired alpha-2-plasmin inhibitor deficiency.ε-氨基己酸治疗急性早幼粒细胞白血病合并获得性α2-纤溶酶抑制剂缺乏症患者
Ann Intern Med. 1986 Dec;105(6):873-7. doi: 10.7326/0003-4819-105-6-873.
6
7
[Blind spots of the diagnostic hemostasis screen].[诊断性止血筛查的盲点]
Ned Tijdschr Geneeskd. 2000 Mar 4;144(10):457-60.
8
Congenital deficiency of alpha 2-plasmin inhibitor in three sisters.三姐妹先天性α2-纤溶酶抑制剂缺乏症
Haemostasis. 1982;11(3):176-84. doi: 10.1159/000214659.
9
Severe hemorrhagic tendency in heterozygous alpha 2-antiplasmin deficiency.
Thromb Res. 1985 Dec 1;40(5):645-51. doi: 10.1016/0049-3848(85)90302-0.
10
Effective therapy with tranexamic acid in a case of chronic disseminated intravascular coagulation with acquired alpha2-antiplasmin deficiency associated with AL amyloidosis.氨甲环酸成功治疗一例与AL淀粉样变性相关的获得性α2-抗纤溶酶缺乏的慢性弥散性血管内凝血病例。
Thromb Haemost. 2009 Dec;102(6):1285-7. doi: 10.1160/TH09-04-0248.
引用本文的文献
1
Neutrophil extracellular trap components increase the expression of coagulation factors.中性粒细胞胞外诱捕网成分增加凝血因子的表达。
Biomed Rep. 2019 Mar;10(3):195-201. doi: 10.3892/br.2019.1187. Epub 2019 Jan 23.
2
Clinical disorders of fibrinolysis: a critical review.纤维蛋白溶解的临床病症：批判性综述。
Blut. 1989 Jul;59(1):1-14. doi: 10.1007/BF00320240.
本文引用的文献
1
The mechanism of clot dissolution by plasmin.纤溶酶溶解血栓的机制。
J Clin Invest. 1959 Jul;38(7):1086-95. doi: 10.1172/JCI103885.
2
3
4
Electroimmuno assay.免疫电泳分析
Scand J Clin Lab Invest Suppl. 1972;124:21-37. doi: 10.3109/00365517209102748.
5
Alpha2-plasmin-inhibitor deficiency (Miyasato disease).
Lancet. 1978;2(8104-5):1334-6. doi: 10.1016/s0140-6736(78)91973-6.
Zotero 插件