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Factor VII Padua: a congenital coagulation disorder due to an abnormal factor VII with a peculiar activation pattern.

作者信息

Girolami A, Fabris F, Dal Bo Zanon R, Ghiotto G, Burul A

出版信息

J Lab Clin Med. 1978 Mar;91(3):387-95.

PMID:627745
Abstract
摘要

相似文献

1
Factor VII Padua: a congenital coagulation disorder due to an abnormal factor VII with a peculiar activation pattern.帕多瓦因子VII：一种由于具有特殊激活模式的异常因子VII导致的先天性凝血障碍。
J Lab Clin Med. 1978 Mar;91(3):387-95.
2
Report of the fifth homozygous patient with factor VII Padua defect.第五例患有凝血因子VII帕多瓦缺陷的纯合子患者报告。
Folia Haematol Int Mag Klin Morphol Blutforsch. 1983;110(3):447-54.
3
The congenital factor VII abnormalities (dysproconvertinemias). The genetic plot thickens.先天性凝血因子VII异常（异常转化素血症）。遗传情况愈发复杂。
Folia Haematol Int Mag Klin Morphol Blutforsch. 1980;107(1):131-6.
4
Congenital factor VII deficiency. Clinical and laboratory characteristics of a newly discovered kindred.先天性因子VII缺乏症。一个新发现家系的临床和实验室特征。
Clin Lab Haematol. 1982;4(2):109-15.
5
Coagulation factors V and VII combined congenital deficiency in a Mexican family: Toledo-Tehuantepec deficiency, a new pathological entity.
Arch Invest Med (Mex). 1985 Jan-Mar;16(1):59-70.
6
Factor VII Padua 1. Another case.
Haemostasis. 1982;11(3):185-8. doi: 10.1159/000214660.
7
[Classical factor VII deficiency: biological and genetic evaluation of 3 families].[经典因子VII缺乏症：3个家族的生物学和遗传学评估]
Sangre (Barc). 1984;29(2):121-32.
8
[Blood coagulation mechanism from the viewpoint of molecular biochemistry: Initiation of blood coagulation].
Rinsho Byori. 1985 Sep;33(9):960-5.
9
Severe factor VII deficiency caused by a novel point mutation (Arg353Pro) combined with a rare Cys22Arg mutation.由一种新型点突变（Arg353Pro）与罕见的Cys22Arg突变共同导致的严重凝血因子VII缺乏症。
Thromb Haemost. 2007 Sep;98(3):687-8.
10
Factor VII Padua 2: another factor VII abnormality with defective ox brain thromboplastin activation and a complex hereditary pattern.凝血因子VII帕多瓦2型：另一种凝血因子VII异常，伴有牛脑凝血活酶激活缺陷及复杂的遗传模式。
Blood. 1979 Jul;54(1):46-53.

引用本文的文献

1
Thromboelastometry as an Ancillary Tool for Evaluation of Coagulation Status after rFVIIa Therapy in a Pregnant Woman with Severe Hypoproconvertinemia-A Case Series and Review of the Literature.血栓弹力描记术作为评价 rFVIIa 治疗严重低蛋白血症孕妇凝血状态的辅助工具：病例系列及文献复习。
Int J Environ Res Public Health. 2022 Sep 1;19(17):10918. doi: 10.3390/ijerph191710918.
2
Compound Heterozygous Factor VII Deficiency c.1025G>A p.(Arg342Gln) With Novel Missense Variant c.194C>G p.(Ala65Gly).复合杂合子因子VII缺乏症，c.1025G>A，p.(精氨酸342谷氨酰胺)，伴有新型错义变体c.194C>G，p.(丙氨酸65甘氨酸)
J Hematol. 2022 Feb;11(1):29-33. doi: 10.14740/jh943. Epub 2022 Feb 26.
3
Biochemical, molecular and clinical aspects of coagulation factor VII and its role in hemostasis and thrombosis.
凝血因子 VII 的生化、分子和临床方面及其在止血和血栓形成中的作用。
Haematologica. 2021 Feb 1;106(2):351-362. doi: 10.3324/haematol.2020.248542.
4
Conformational Changes of Congenital FVII Variants with Defective Binding to Tissue Factor ARG304GLN (FVII Padua), ARG 304TRP (FVII Nagoya) and ARG79GLN (FVII Shinjo or Tondabayashi).与组织因子结合缺陷的先天性FVII变体（ARG304GLN（FVII帕多瓦）、ARG 304TRP（FVII名古屋）和ARG79GLN（FVII新庄或富田林））的构象变化
Int J Biomed Sci. 2013 Dec;9(4):185-93.
5
Congenital FVII deficiency and thrombotic events after replacement therapy.先天性 FVII 缺乏症和替代治疗后的血栓事件。
J Thromb Thrombolysis. 2011 Oct;32(3):362-7. doi: 10.1007/s11239-011-0603-8.
6
Associated prothrombotic conditions are probably responsible for the occurrence of thrombosis in almost all patients with congenital FVII deficiency. Critical review of the literature.相关的促血栓形成情况可能是几乎所有先天性 FVII 缺乏症患者发生血栓形成的原因。文献的批判性回顾。
J Thromb Thrombolysis. 2010 Aug;30(2):172-8. doi: 10.1007/s11239-009-0435-y.
7
Hemophilia B with associated factor VII deficiency: a distinct variant of hemophilia B with low factor VII activity and normal factor VII antigen.伴有因子VII缺乏的B型血友病：一种具有低因子VII活性和正常因子VII抗原的B型血友病独特变体。
Blut. 1980 Apr;40(4):267-73. doi: 10.1007/BF01080186.
8
Demonstration of a double hereditary pattern for congenital afibrinogenemia.先天性无纤维蛋白原血症的双遗传模式证明。
Blut. 1981 Oct;43(4):249-56. doi: 10.1007/BF00320454.
9
Further studies on factor VII Padua defect: the report of the fourth homozygous patient from the same valley.
Blut. 1982 Jun;44(6):363-9. doi: 10.1007/BF00319920.
10
Incidence, significance, and subtypes of hemophilia BM in a large population of hemophilia B patients.大量B型血友病患者中B型血友病携带者的发病率、意义及亚型
Blut. 1982 Jan;44(1):41-9. doi: 10.1007/BF00320685.