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1
Fitzgerald Trait: Deficiency of a Hitherto Unrecognized Agent, Fitzgerald Factor, Participating in Surface-Mediated Reactions of Clotting, Fibrinolysis, Generation of Kinins, and the Property of Diluted Plasma Enhancing Vascular Permeability (PF/Dil).菲茨杰拉德特质:凝血、纤维蛋白溶解、激肽生成的表面介导反应以及稀释血浆增强血管通透性(PF/Dil)的 hitherto 尚未被识别的因子、菲茨杰拉德因子的缺乏。
J Clin Invest. 1975 May;55(5):1082-9. doi: 10.1172/JCI108009.
2
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.
3
Kininogen deficiency in Fitzgerald trait: role of high molecular weight kininogen in clotting and fibrinolysis.菲茨杰拉德性状中的激肽原缺乏:高分子量激肽原在凝血和纤维蛋白溶解中的作用。
J Lab Clin Med. 1976 Feb;87(2):327-37.
4
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.
5
Studies on Fletcher trait and Fitzgerald trait. A rare chance to disclose body's defense reactions against injury.弗莱彻特质和菲茨杰拉德特质研究。揭示身体对损伤的防御反应的罕见机会。
Thromb Haemost. 2010 Nov;104(5):867-74. doi: 10.1160/TH10-01-0058. Epub 2010 Sep 13.
6
The first cases of Fitzgerald factor deficiency in the Orient: three cases in one family.东方首例菲茨杰拉德因子缺乏症:一个家族中的三例病例。
Acta Haematol. 1980;63(2):107-13. doi: 10.1159/000207379.
7
New congenital deficiency of high molecular weight kininogen and prekallikrein (Fitzgerald trait). Study of response to DDAVP and venous occlusion.高分子量激肽原和前激肽释放酶新的先天性缺乏症(菲茨杰拉德特质)。对去氨加压素和静脉闭塞反应的研究。
Haematologia (Budap). 1986;19(1):41-8.
8
Evidence that Fitzgerald factor counteracts inhibition by kaolin or ellagic acid of the amidolytic properties of a plasma kallikrein.有证据表明,菲茨杰拉德因子可抵消高岭土或鞣花酸对血浆激肽释放酶酰胺分解特性的抑制作用。
Blood. 1976 Feb;47(2):243-51.
9
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.
10
Clinical and physiologic studies of two siblings with prekallikrein (Fletcher factor) deficiency.两名前激肽释放酶(弗莱彻因子)缺乏症同胞的临床和生理学研究。
Am J Med. 1976 May 10;60(5):654-64. doi: 10.1016/0002-9343(76)90500-3.

引用本文的文献

1
The complex role of kininogens in hereditary angioedema.激肽原在遗传性血管性水肿中的复杂作用。
Front Allergy. 2022 Aug 3;3:952753. doi: 10.3389/falgy.2022.952753. eCollection 2022.
2
Anti-HK antibody reveals critical roles of a 20-residue HK region for Aβ-induced plasma contact system activation.抗 HK 抗体揭示了 20 残基 HK 区域对 Aβ诱导的血浆接触系统激活的关键作用。
Blood Adv. 2022 May 24;6(10):3090-3101. doi: 10.1182/bloodadvances.2021006612.
3
The contact activation system and vascular factors as alternative targets for Alzheimer's disease therapy.接触激活系统和血管因素作为阿尔茨海默病治疗的替代靶点。
Res Pract Thromb Haemost. 2021 May 3;5(4):e12504. doi: 10.1002/rth2.12504. eCollection 2021 May.
4
Hereditary angioneurotic oedema: characterization of plasma kinin and vascular permeability-enhancing activities.遗传性血管性水肿:血浆激肽及血管通透性增强活性的特征
Clin Exp Immunol. 1994 Jan;95(1):22-8. doi: 10.1111/j.1365-2249.1994.tb06009.x.
5
Function and immunochemistry of prekallikrein-high molecular weight kininogen complex in plasma.血浆中前激肽释放酶-高分子量激肽原复合物的功能与免疫化学
J Clin Invest. 1980 Feb;65(2):413-21. doi: 10.1172/JCI109684.
6
Congenital deficiency in plasma kallikrein and kininogens in the brown Norway rat.棕色挪威大鼠血浆激肽释放酶和激肽原的先天性缺乏
Experientia. 1980 May 15;36(5):586-7. doi: 10.1007/BF01965817.
7
The concentration of high molecular weight kininogen antigen in homogenates of various human tissues.各种人体组织匀浆中高分子量激肽原抗原的浓度。
Experientia. 1980 Mar 15;36(3):354-6. doi: 10.1007/BF01952323.
8
Kinetic analysis of the interaction of human tissue kallikrein with single-chain human high and low molecular weight kininogens.人组织激肽释放酶与单链人高分子量和低分子量激肽原相互作用的动力学分析
Proc Natl Acad Sci U S A. 1983 Jul;80(13):3928-32. doi: 10.1073/pnas.80.13.3928.
9
Synthesis and release of Hageman factor (Factor XII) by the isolated perfused rat liver.离体灌注大鼠肝脏中哈格曼因子(凝血因子 XII)的合成与释放
J Clin Invest. 1983 Sep;72(3):948-54. doi: 10.1172/JCI111066.
10
Synthesis and secretion of alpha 2-plasmin inhibitor by established human liver cell lines.已建系的人肝细胞系对α2-纤溶酶抑制剂的合成与分泌
Proc Natl Acad Sci U S A. 1982 Sep;79(18):5684-7. doi: 10.1073/pnas.79.18.5684.

本文引用的文献

1
Kinin formation and fibrinolysis in human plasma.人血浆中的激肽形成与纤维蛋白溶解
J Physiol. 1963 May;166(3):514-29. doi: 10.1113/jphysiol.1963.sp007120.
2
[Role of the contact factor (Hageman factor) in fibrinolysis].[接触因子(哈格曼因子)在纤维蛋白溶解中的作用]
Thromb Diath Haemorrh. 1959 Sep 1;3:593-603.
3
Assay and properties of serum inhibitor of C'l-esterase.C1酯酶血清抑制剂的测定与特性
Proc Soc Exp Biol Med. 1959 Aug-Sep;101:608-11. doi: 10.3181/00379727-101-25034.
4
Enzyme-like globulins from serum reproducing the vascular phenomena of inflammation. I. An activable permeability factor and its inhibitor in guinea-pig serum.血清中类似酶的球蛋白再现炎症的血管现象。I. 豚鼠血清中的一种可激活的通透性因子及其抑制剂。
Br J Exp Pathol. 1955 Feb;36(1):71-81.
5
THE INDUCTION OF PERMEABILITY-INCREASING ACTIVITY IN HUMAN PLASMA BY ACTIVATED HAGEMAN FACTOR.活化的哈格曼因子诱导人血浆中通透性增加活性
Br J Exp Pathol. 1964 Jun;45(3):328-45.
6
A micromethod for determination of bradykininogen under several conditions.一种在多种条件下测定缓激肽原的微量方法。
Ann N Y Acad Sci. 1963 Feb 4;104:77-89. doi: 10.1111/j.1749-6632.1963.tb17654.x.
7
The purification of activated Hageman factor (activated factor XII).活化的哈格曼因子(活化的因子XII)的纯化。
Biochemistry. 1962 Nov;1:967-75. doi: 10.1021/bi00912a005.
8
Evidence for a new plasma thromboplastin factor. I. Case report, coagulation studies and physicochemical properties.一种新的血浆凝血活酶因子的证据。I. 病例报告、凝血研究及物理化学性质。
Blood. 1965 Nov;26(5):521-32.
9
Kinins: possible mediators of neonatal circulatory changes in man.激肽:人类新生儿循环变化的潜在介质
J Clin Invest. 1968 Jun;47(6):1295-302. doi: 10.1172/JCI105821.
10
Immunologic differentiation of classic hemophilia (factor 8 deficiency) and von Willebrand's dissase, with observations on combined deficiencies of antihemophilic factor and proaccelerin (factor V) and on an acquired circulating anticoagulant against antihemophilic factor.经典血友病(因子Ⅷ缺乏症)和血管性血友病的免疫学分型,以及关于抗血友病因子和加速素(因子Ⅴ)联合缺乏症和一种获得性抗血友病因子循环抗凝剂的观察
J Clin Invest. 1971 Jan;50(1):244-54. doi: 10.1172/JCI106480.

菲茨杰拉德特质:凝血、纤维蛋白溶解、激肽生成的表面介导反应以及稀释血浆增强血管通透性(PF/Dil)的 hitherto 尚未被识别的因子、菲茨杰拉德因子的缺乏。

Fitzgerald Trait: Deficiency of a Hitherto Unrecognized Agent, Fitzgerald Factor, Participating in Surface-Mediated Reactions of Clotting, Fibrinolysis, Generation of Kinins, and the Property of Diluted Plasma Enhancing Vascular Permeability (PF/Dil).

机构信息

Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106.

出版信息

J Clin Invest. 1975 May;55(5):1082-9. doi: 10.1172/JCI108009.

DOI:10.1172/JCI108009
PMID:16695963
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC301855/
Abstract

The prolonged partial thromboplastin time observed in the plasma of a 71-yr-old asymptomatic man was related to the deficiency of a hitherto unrecognized agent. The patient's plasma also exhibited impaired surface-mediated fibrinolysis and esterolytic activity and impaired generation of kinins and of the property enhancing vascular permeability designated PF/Dil. The patient's plasma contained normal amounts of all known clotting factors except Fletcher factor (a plasma prekallikrein) which was present at a concentration of 10-15% of pooled normal plasma. Fletcher trait plasma, however, contained normal amounts of the agent missing from the patient's plasma and corrected the defects in clotting, fibrinolysis, and vascular permeability. Fletcher trait plasma was less effective in correcting generation of kinins and esterolytic activity, presumably because of the patient's partial deficiency of prekallikrein. The site of action of the factor deficient in the patient's plasma appeared to be subsequent to the activation of Hageman factor and plasma prekallikrein. A fraction of normal plasma, devoid of other clotting factors, corrected the defect in clotting in the patient's plasma; a similar fraction of the patient's plasma did not correct this abnormality. No evidence yet exists pointing to the familial nature of the patient's defect. Tentatively, the patient's disorder may be referred to by his surname as Fitzgerald trait, and the agent apparently deficient in his plasma as Fitzgerald factor.

摘要

一位 71 岁无症状男性的血浆中出现了延长的部分凝血活酶时间,这与一种以前未被识别的因子缺乏有关。该患者的血浆还表现出表面介导的纤维蛋白溶解和酯酶活性受损,以及激肽生成和血管通透性增强因子(PF/Dil)生成受损。该患者的血浆中除 Fletcher 因子(血浆前激肽释放酶)外,含有所有已知凝血因子的正常量,Fletcher 因子的浓度为正常混合血浆的 10-15%。然而,Fletcher 特征性血浆中含有患者血浆中缺失的因子的正常量,并纠正了凝血、纤维蛋白溶解和血管通透性的缺陷。Fletcher 特征性血浆在纠正激肽生成和酯酶活性方面的效果较差,可能是因为患者存在部分前激肽释放酶缺乏。患者血浆中缺乏的因子的作用部位似乎在 Hageman 因子和血浆前激肽释放酶激活之后。正常血浆的一部分,没有其他凝血因子,纠正了患者血浆中的凝血缺陷;患者血浆的类似部分不能纠正这种异常。目前尚无证据表明患者的缺陷具有家族性质。暂时可以将患者的疾病称为 Fitzgerald 特征,而其血浆中明显缺乏的因子称为 Fitzgerald 因子。