Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that leads to mucocutaneous telangiectasias, epistaxis, and gastrointestinal bleeding. Depending on the severity and manifestation of the disease, various therapeutic modalities have been used, from local bleeding control to surgery or concomitant drug therapy. Several articles under review have presented guidelines f...

Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu syndrome is an inherited disorder characterized by vascular dysplasias leading to hemorrhages. If affects approximately 1 in 10,000 Caucasian people. The most common presentation chronic and recurrent epistaxis whereas bleeding from other sites can lead life-threatening complications.

OBJECTIVE Upper gastrointestinal (UGI) bleeding is one of the most life-threatening complications, in up to 25% of persons with hemophilia (PWH). Recurrent bleeding is common and can be caused by the Helicobacter pylori infection. Our aim was to evaluate the role of H. pylori infection in UGI bleeding in PWH. MATERIAL AND METHODS Ninety patients with hereditary bleeding disorders, 30 patients...

conclusions the case was fully investigated and diagnosed as a case with factor xiii deficiency causing such uncontrolled bleeding after extraction of a tooth. introduction factor xiii deficiency is a relatively rare hereditary bleeding disorder, which is usually found during infancy or early childhood. this condition may involve both genders within different races in an equal manner. its incid...

Blood Transfus 2011; 9 Suppl 2:s3-s8 DOI 10.2450/2011.002S © SIMTI Servizi Srl von Willebrand factor (VWF) is an adhesive and multimeric glycoprotein that found its historical origin in 1924, when the Finnish physician Erik von Willebrand first reported a family with a serious hereditary bleeding affecting consanguineous families. The proband was a five years old girl with severe bleeding since...

Cystic fibrosis (CF) is a hereditary disease of mucous and sweat glands, which affects the respiratory and gastrointestinal systems. Herein, we describe a 3-month-old girl with a history of recurrent episodes of urinary tract infections that required hospitalization. She was referred to our center at the age of three months, with massive gastroesophageal variceal bleeding. In physical examinati...

Hereditary Hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber disease, is a vascular anomaly characterized by multiple dilations of skin and mucosa capillaries and venules. It is an autosomal dominant disease, equally distributed between both genders and its incidence is of 1-2/100.000 inhabitants.1 Bleeding may occur in numerous places; however, epistaxis is the most common, present in 90%...

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...

Hereditary thrombocytopenias are rare bleeding disorders, which cause a deficiency of platelets in early infancy. This group of disorders is sometimes associated with abnormal phenotypes, like absence of radius. Diagnosis of this type of thrombocytopenia is usually difficult; other causes of thrombocytopenia, such as immune disorders and infections, must be ruled out. The symptoms of hereditary...