Purpose: A 41-year-old woman with familial antithrombin-III deficiency was admitted to our hospital for patch closure of an atrial septal defect. Antithrombin-III activity was 43% and its antigen level was 12.2 mg/dl, was diagnosed so type I antithrombin-III deficiency was diagnosed.
Methods: A dose of 2500 U of antithrombin-III concentrate was administered at 1 day before surgery, 1 hour before surgery, and 1 day after surgery. Heparinazation was performed at 200 IU/kg and the activated clotting time increased from a baseline of 140s to 622s. After establishing cardiopulmonary bypass and cardioplegic arrest, closure of the septal defect was done with a pericardial patch.
Results: The activated clotting time was maintained at more than 400s during cardiopulmonary bypass. There were no intraoperative complications and the postoperative course was uneventful.
Conclusion: In patients with familial antithrombin-III deficiency, administration of antithrombin-III concentrate is effective when cardiopulmonary bypass is required.
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http://dx.doi.org/10.5761/atcs.cr.12.01981 | DOI Listing |
Thromb Res
January 2025
Department of Neurology, Fujian Institute of Geriatrics, Fujian Medical University Union Hospital, Fuzhou, Fujian, China. Electronic address:
Background: Protein S deficiency is a rare inherited disease. We report the case of a young man who unexpectedly developed isolated cortical vein thrombosis (ICoVT) associated with a novel PROS1 mutation.
Methods: Clinical symptoms were recorded, and physical examinations conducted.
J Med Case Rep
January 2025
Headache Department, Iranian Center of Neurological Research, Neuroscience Institute, Tehran University of Medical Sciences, Tehran, Iran.
Background: Idiopathic intracranial hypertension (IIH) is a condition where the pressure of the cerebrospinal fluid in the brain increases without a known cause. It typically affects adults but can also occur in adolescents and children, although it is less common. Numerous elements, including coagulopathy, have been documented in previous cases as potential etiological factors of IIH.
View Article and Find Full Text PDFOrphanet J Rare Dis
December 2024
Thrombosis Research Center, Beijing Jishuitan Hospital, Capital Medical University, Xicheng District, Beijing, 100035, China.
Background: Identification of mutations in the SERPINC1 has illuminated the intricate pathways underlying antithrombin (AT) deficiency. Our group identified a variation in the SERPINC1 gene (c.964 A > T, p.
View Article and Find Full Text PDFArterioscler Thromb Vasc Biol
January 2025
Division of Cardiothoracic Anesthesiology, Department of Anesthesiology and Critical Care, Perelman School of Medicine at the University of Pennsylvania, Philadelphia (L.K., N.K.T.).
Background: Andexanet alfa (andexanet) is the only Food and Drug Administration-approved antidote for direct FXa (factor Xa) inhibitors but has been reported to cause resistance to unfractionated heparin (UFH). This has delayed anticoagulation for procedures requiring cardiopulmonary bypass. The mechanism, andexanet and UFH dose dependence, and thrombotic risk of andexanet-associated heparin resistance are unknown.
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