BACKGROUND AND OBJECTIVES Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is an autosomal dominant disorder characterized by mucocutaneous and visceral vascular dysplasia associated with frequent episodes of epistaxis and gastrointestinal bleeding. The objective of this report was to describe the anesthesia of a patient with this syndrome. CASE REPORT A ...

Orthodontic treatment is fully consistent with general health and it also improves quality-of life along with psychological considerations. Haemophilia is a disorder in which due to deficiency of some coagulation factors. Hereditary deficiency of coagulation factors has caused 90% of hereditary diseases: Haemophilia A, Haemophilia B and von Willebrand’s. Two issues need to be considered in the ...

factor v (fv) deficiency is a rare bleeding disorder (rbd) that inherit in autosomal recessive manner. diagnosis of fv deficiency (fvd) is made by routine coagulation tests, fv activity and molecular analysis. in patients with fvd, routine coagulation tests including activated partial thromboplastin time (aptt), prothrombin time (pt) and evenbleeding time (bt) are prolongedwhile thrombin time (...

Bleeding and bruising are common symptoms in the primary care setting. The patient history can help determine whether the bruising or bleeding is abnormal. The International Society on Thrombosis and Hemostasis has developed a bleeding assessment tool that can be used to indicate possible pathology. A family history of bleeding problems may suggest a hereditary coagulation defect. Such a histor...

background: the most common polymorphisms identified in the methylenetetrahydrofolate reductase (mthfr) gene, c677t and a1298c lead to defective activity of this enzyme and increase the risk of venous and arterial thrombosis. there are limited investigations regarding the effects of thrombogenic polymorphisms on the clinical phenotypes of rare hereditary hemorrhagic disorders like glanzmann's t...

Invasive procedures for patients with bleeding disorders require planning on the part of the health care team. The patient population affected involves those with hereditary bleeding disorders, such as von Willebrand disease or hemophilia; in addition, patients who use antithrombotic drugs must be considered and their care managed. The choice of treatment depends on a number of factors, includi...

discussions as one ich patient whose pt and aptt suggest a coagulation disorder secondary to vitamin k deficiency or coagulation factor deficiency, unresponsiveness to vitamin k therapy should be useful to take fx deficiency into consideration. background inborn factor x deficiency (fxd) is a very rare (1: 500,000) hereditary coagulation disorder, which is characterized by clinical manifestatio...

Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by uncontrolled multisystem angiogenesis with epistaxis, gastrointestinal bleeding, iron-deficiency anemia, and arteriovenous malformations, and is often associated with increased levels of vascular endothelial growth factor (VEGF). Bevacizumab, a VEGF inhibitor, reduces epistaxis, telangiectasias, and iron-deficien...