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[一种作用于“接触”水平的新型凝血因子遗传性缺乏:“弗洛热阿克”因子]

[Hereditary deficiency of a new coagulation factor acting at the "contact" level: the "Flaujeac" factor].

作者信息

Lacombe M J

出版信息

C R Acad Hebd Seances Acad Sci D. 1975 Feb 24;280(8):1039-41.

PMID:809188
Abstract

The observation of a coagulation defect which has been observed in an adult French woman is reported. This deficiency which induces no bleeding tendency is characterized by a considerable lengthening of the activated Partial Thromboplastin Time and an abnormality of the fibrinolysis activation. Circulating anticoagulant is absent. The levels of HF, PTA, AHF, PTC are normal and mutual correction with Fletcher factor deficient plasma is obtained. This result leads to the conclusion that patient's plasma is dificient in a hitherto undescribed coagulation factor. This factor seems to interfere, in vitro process, at the level of glass activation, and after the activation of Hageman factor.

摘要

报告了一名成年法国女性出现凝血缺陷的观察情况。这种无出血倾向的缺陷表现为活化部分凝血活酶时间显著延长以及纤维蛋白溶解活化异常。未发现循环抗凝物质。HF、PTA、AHF、PTC水平正常,且与 Fletcher 因子缺乏血浆相互校正。这一结果得出结论,患者血浆中缺乏一种迄今未描述的凝血因子。该因子似乎在体外过程中,在玻璃活化水平以及接触因子活化后发挥干扰作用。

相似文献

1
[Hereditary deficiency of a new coagulation factor acting at the "contact" level: the "Flaujeac" factor].[一种作用于“接触”水平的新型凝血因子遗传性缺乏:“弗洛热阿克”因子]
C R Acad Hebd Seances Acad Sci D. 1975 Feb 24;280(8):1039-41.
2
A hitherto undescribed plasma factor acting at the contact phase of blood coagulation (Flaujeac factor): case report and coagulation studies.一种作用于血液凝固接触阶段的此前未被描述的血浆因子(弗洛热阿克因子):病例报告及凝血研究
Blood. 1975 Nov;46(5):761-8.
3
Prekallikrein deficiency in man.人类前激肽释放酶缺乏症。
J Exp Med. 1973 Dec 1;138(6):1345-55. doi: 10.1084/jem.138.6.1345.
4
Severe Fletcher factor (plasma prekallikrein) deficiency with partial deficiency of Hageman factor (factor XII): report of a case with observation on in vivo and in vitro leukocyte chemotaxis.重度弗莱彻因子(血浆前激肽释放酶)缺乏伴哈格曼因子(因子Ⅻ)部分缺乏:1例报告及体内和体外白细胞趋化性观察
Am J Hematol. 1982 May;12(3):261-70. doi: 10.1002/ajh.2830120308.
5
Interactions among Hageman factor (HG, Factor XII), plasma thromboplastin antecedent (PTA, Factor XI), plasma prekallikrein (PK, Fletcher factor) and high molecular weight kininogen (HMW-K, Fitzgerald factor) in blood coagulation.血液凝固过程中,接触因子(HG,凝血因子XII)、血浆凝血活酶前体(PTA,凝血因子XI)、血浆前激肽释放酶(PK,弗莱彻因子)和高分子量激肽原(HMW-K,菲茨杰拉德因子)之间的相互作用。
Adv Exp Med Biol. 1979;120B:61-70.
6
Flaujeac trait. Deficiency of human plasma kininogen.弗洛热阿克性状。人血浆激肽原缺乏。
J Clin Invest. 1975 Dec;56(6):1663-72. doi: 10.1172/JCI108248.
7
Fletcher factor deficiency. A diminished rate of Hageman factor activation caused by absence of prekallikrein with abnormalities of coagulation, fibrinolysis, chemotactic activity, and kinin generation.弗莱彻因子缺乏症。由于缺乏前激肽释放酶导致哈格曼因子激活速率降低,伴有凝血、纤维蛋白溶解、趋化活性和激肽生成异常。
J Clin Invest. 1974 Feb;53(2):622-33. doi: 10.1172/JCI107597.
8
Prekallikrein (Fletcher factor) deficiency.前激肽释放酶(弗莱彻因子)缺乏症。
Ann Clin Lab Sci. 1985 Jul-Aug;15(4):279-85.
9
Williams trait. Human kininogen deficiency with diminished levels of plasminogen proactivator and prekallikrein associated with abnormalities of the Hageman factor-dependent pathways.威廉姆斯特征。人类激肽原缺乏,纤溶酶原激活物和前激肽释放酶水平降低,与哈格曼因子依赖性途径异常相关。
J Clin Invest. 1975 Dec;56(6):1650-62. doi: 10.1172/JCI108247.
10
The role of HF cofactor in the Hageman factor-dependent fibrinolytic mechanism.HF辅因子在接触因子依赖性纤溶机制中的作用。
Acta Haematol. 1981;66(4):257-63. doi: 10.1159/000207131.

引用本文的文献

1
Flaujeac factor deficiency. Reconstitution with highly purified bovine high molecular weight-kininogen and delineation of a new permeability-enhancing peptide released by plasma kallikrein from bovine high molecular weight-kininogen.弗洛热亚克因子缺乏症。用高度纯化的牛高分子量激肽原进行重建,并鉴定血浆激肽释放酶从牛高分子量激肽原释放的一种新的通透性增强肽。
J Clin Invest. 1976 Dec;58(6):1395-406. doi: 10.1172/JCI108595.
2
Flaujeac trait. Deficiency of human plasma kininogen.弗洛热阿克性状。人血浆激肽原缺乏。
J Clin Invest. 1975 Dec;56(6):1663-72. doi: 10.1172/JCI108248.