Cases reported "Factor XII Deficiency"

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1/4. Huge left atrial thrombus with mitral stenosis in congenital factor xii deficiency.

    factor xii deficiency has been reported to be a risk factor for thromboembolism as a result of inactivation of fibrinolysis. We describe a case of a huge left atrial thrombus with mitral stenosis, which was successfully removed surgically in a Factor XII deficient patient.
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keywords = thromboembolism
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2/4. factor xii deficiency and pregnancy.

    A 19-year-old primigravida with known factor xii deficiency (prepregnant factor XII level of 21%) presented with placental abruption and preterm labor at 26 weeks' gestation. A healthy 925-g female infant was born by spontaneous vaginal delivery. The mother had no postpartum hemorrhage or further complications, and the infant demonstrated no intracranial or other forms of hemorrhage up to 70 days of age. The infant's factor XII level was 34% (normal for her age). There are only two previous reports of factor xii deficiency in pregnancy cited in the English literature, and both were uncomplicated. In view of the risk of thromboembolic complications in nonpregnant individuals with factor xii deficiency, pregnant women with a prolonged activated partial thromboplastin time and no lupus anticoagulant or anticardiolipin antibody syndrome should also be investigated for deficiencies of factors VIII, IX, and XII. These patients should be given the appropriate counseling and should be monitored for features of thromboembolism if factor xii deficiency is confirmed.
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keywords = thromboembolism
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3/4. Clinical implications of factor xii deficiency.

    A patient with known factor xii deficiency underwent extraction of four impacted third molars. Significant preoperative laboratory values included a partial thromboplastin time (PTT) of greater than 100 seconds and a factor XII level of less than 1%. The third molars were removed without any significant intraoperative or postoperative bleeding. Factor XII not only has an important function in the initiation of the intrinsic pathway of the coagulation cascade, but it also plays a significant role in complement activation, kinin generation, and fibrinolysis. It would seem that a deficiency in this factor would have widespread clinical implications. In fact, the only clinical significance seems to be a predisposition to thromboembolism in factor XII deficient patients.
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keywords = thromboembolism
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4/4. Thrombus formation on transcatheter ASD occluder device in a patient with coagulation factor xii deficiency.

    Transcatheter occlusion of cardiac defects has become an effective and less invasive alternative to open heart surgery. Thromboembolic complications are rare events, after both surgical and transcatheter closure of atrial septal defects [Galal et al.: Eur heart J 15:1381-1384, 1994]. We report on a case of thrombus formation on the atrial septal defect occluder system (ASDOS) [Sievert et al.: Cathet Cardiovasc Diagn 36:232-240, 1995; Hausdorf et al.: heart 75:83-88, 1996]. Two days after transcatheter occlusion, the patient suffered an acute stroke due to embolism despite anticoagulation with intravenous heparin. A coagulation disorder with reduced factor XII concentration was deduced as the likely cause. Repeated transesophageal echocardiographic (TEE) studies revealed an involution of the intracardial thrombus within weeks of subsequent anticoagulatory treatment. First off, this case shows that patients with factor xii deficiency are at risk for thromboembolism. Second, it again clarifies that even large amounts of intraartrial thrombotic material may not be seen by transthoracic echocardiography (TTE) and underscores the necessity of performing TEE. Screening patients for coagulation disorders (Quick's value (Q), partial thromboplastin time (PTT)) before they are selected for treatment with thrombogenic devices is indispensable. With regard to their personal history (earlier thromboembolism) and the result of this screening (e.g., prolongation of PTT), quantitative determination of coagulation factors is reasonable. If patients endangered by thromboembolic complications nevertheless undergo ASD occlusion procedures, anticoagulation monitoring requires exceptional attention. Furthermore, it is recommended that TEE should be carried out in these patients 2 days after treatment, since transthoracic echocardiography (TTE) might be unable to detect thrombus formation on the device.
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ranking = 2
keywords = thromboembolism
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