Hereditary benign telangiectasia is an uncommon idiopathic skin disorder characterized by generalized telangiectases and angiomatous lesions of the skin. The diagnosis should be suspected in patients with such cutaneous lesions, positive family history, no associated bleeding problems, and no mucosal involvement. We present six cases of hereditary benign telangiectasia in three generations of o...

Background: Data on the frequency of hereditary bleeding disorders (HBDs) and associated mortality and morbidities during a long-term follow-up from Iran are scarce. Objective: This study evaluated the epidemiologic features among patients with HBD in one of the largest referral centers in southern Iran. Methods: In this cross-secti...

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...

Hereditary haemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) is an autosomal dominant vascular disorder, manifesting with telangiectases and bleeding in different parts of the body. We report a patient who presented with bleeding from various sites.

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.

Background: Congenital hepatic fibrosis (CHF) is an autosomal hereditary disorder affecting the porto-biliary system. It is a rare hereditary disorder often presenting in childhood or adolescence with hepatomegaly, splenomegaly, and gastrointestinal bleeding. A timely diagnosis of organomegalies by sonography can prevent esophageal varices. Liver transplantation is now the only...

Hereditary hemorrhagic telangiectasia (HHT), Osler-Weber-Rendu Syndrome, is an uncommon disease but may be present in many people who remain undiagnosed. It is an autosomal dominant disorder characterized by multiple arteriovenous malformations (AVMs) and telangiectases that affect multiple organ systems. Hereditary hemorrhagic telangiectasia patients have a propensity for bleeding, especially ...

background and aims: human t-lymphotropic virus (htlv) is a human retrovirus which has been known to cause adult t-cell leukemia/lymphoma and some other inflammatory disorders. patients with hereditary bleeding diseases are at high risk for these viruses. in this study, we evaluated serological htlv-i/ii infection among these patients in west azerbaijan of iran. material and methods: we studied...

Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does however not always have to be caused by angioedema but can relate to other concomitant disorders. In th...