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Factor XI Deficiency and a Platelet Defect
Pathophysiology of Haemostasis and Thrombosis, 1983A patient with a lifelong bleeding tendency of moderate severity was found to have both factor XI deficiency and a long bleeding time. There was a significant deficiency of platelet factor 3, but other parameters of platelet function were normal.
M, Winter, J, Needham, P, Barkhan
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Inheritance and bleeding in factor XI deficiency
British Journal of Haematology, 1988A study of 20 Jewish and four non‐Jewish kindreds transmitting factor XI deficiency (164 individuals) confirmed inheritance to be autosomal with severe deficiency in homozygotes (mean factor XI level 3.8 u/dl, SD 2.91) and partial deficiency in heterozygotes (mean factor XI level 57 u/dl, SD 10.42; normal mean factor XI level 96 u/dl, SD 11.6).
P H, Bolton-Maggs +4 more
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New observations on factor XI deficiency
Haemophilia, 2004Summary. Factor (F) XI is an injury‐related bleeding tendency that commonly occurs when trauma involves tissues rich in fibronolytic activators. Severe FXI deficiency is defined when the activity of FXI in plasma is less than 15 U dL−1. The disorder is inherited as an autosomal recessive trait manifesting in homozygotes or compound heterozygotes, and ...
O, Salomon, U, Seligsohn
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Pulmonary Valvulotomy in Factor XI Deficiency
The Thoracic and Cardiovascular Surgeon, 1983This is the first reported case in the English literature of Factor XI deficiency in a child from India. He underwent open-heart surgery and pulmonary valvulotomy without excessive or delayed hemorrhage using plasma infusion to sustain Factor XI levels at about 40% of normal before, and for 10 days after surgery.
T E, Williams +6 more
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Congenital Factor XI Deficiency: An Update
Seminars in Thrombosis and Hemostasis, 2013Severe factor XI (FXI) deficiency is an injury-related bleeding disorder, common in Ashkenazi Jews (with two mutations prevailing), but rare worldwide (with heterogeneous mutations). In the past two decades, more than 220 mutations in the FXI gene have been reported in patients with FXI deficiency, of which 7 showed a founder effect.
Stefano, Duga, Ophira, Salomon
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Factor XI Deficiency and Obstetrical Anesthesia
Anesthesia & Analgesia, 2009Factor XI (FXI) deficiency is a rare inherited coagulation disorder associated with prolonged activated partial thromboplastin time. The severity of bleeding often does not correlate with plasma factor levels. We reviewed the medical and anesthetic records of 13 parturients with FXI deficiency that presented for delivery.
Amarjeet, Singh +3 more
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2018
This chapter outlines the pathophysiology, clinical presentation, inheritance, and incidence of factor XI deficiency. The chapter describes the interaction with pregnancy and discusses the medical and anesthetic management of pregnant women with factor XI deficiency, including treatment of acute hemorrhage.
James P. R. Brown, Joanne Douglas
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This chapter outlines the pathophysiology, clinical presentation, inheritance, and incidence of factor XI deficiency. The chapter describes the interaction with pregnancy and discusses the medical and anesthetic management of pregnant women with factor XI deficiency, including treatment of acute hemorrhage.
James P. R. Brown, Joanne Douglas
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Factor XI deficiency in animal models
Journal of Thrombosis and Haemostasis, 2009The blood coagulation system forms fibrin to limit blood loss from sites of injury, but also contributes to occlusive diseases such as deep vein thrombosis, myocardial infarction, and stroke. In the current model of a coagulation balance, normal hemostasis and thrombosis represent two sides of the same coin; however, data from coagulation factor XI ...
T, Renné +6 more
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A murine model of factor XI deficiency
Blood Coagulation & Fibrinolysis, 1997To facilitate investigations into the physiologic and pathologic roles of factor XI, we have developed a murine model of severe factor XI deficiency using the technique of homologous recombination in embryonic stem cells. The factor XI gene was disrupted by introducing a neomycin phosphotransferase gene into the fifth exon.
D, Gailani, N M, Lasky, G J, Broze
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Factor XI deficiency—resolving the enigma?
Hematology, 2009Abstract The management of factor XI deficiency is not straightforward for three reasons: firstly, the role of this factor in the coagulation pathway is not clearly understood; secondly, the bleeding tendency, although mild, is unpredictable and does not clearly relate to the factor XI level; and thirdly, all treatment products, although
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