A 53-year-old man presented with sudden onset left-sided weakness. His medical history included hereditary hemorrhagic telangiectasia with recurrent epistaxis. On examination, he was hypoxemic at rest and had left-sided flaccid hemiparesis. He was noted to have multiple lower lip telangiectasias (Figure 1). Computed tomography of chest revealed bilateral segmental and subsegmental pulmonary emb...

Defective paracrine Transforming Growth Factor-β (TGF-β) signaling between endothelial cells and the neighboring mural cells have been thought to lead to the development of vascular lesions that are characteristic of Hereditary Hemorrhagic Telangiectasia (HHT). This review highlights recent progress in our understanding of TGF-β signaling in mural cell recruitment and vessel stabilization and h...

The Brain in Hereditary Hemorrhagic Telangiectasia • While neurological symptoms are often mentioned in reports of families with hereditary hemorrhagic telangiectasia (HHT) and are frequently assumed to be due to vascular anomalies of the central nervous system, documentation of such anomalies is surprisingly rare. Two cases of surgically treated symptomatic cerebral vascular malformations in H...

Hereditary hemorrhagic telangiectasia (HHT) is a familial angiodysplastic disorder. Dermal, mucosal, and visceral vascular lesions of this disorder are well known. However, central nervous system (CNS) manifestations, occurring in as many as one-third of patients, have not been well appreciated until recently. The etiology of neurologic symptomatology includes hypoxemia or ischemia secondary to...

472 Communications to the Editor 1 Glauser FL, Fairman RP The uncertain role of the neutrophil in increased permeability pulmonary edema. Chest 1985; 88:601-07 2 Dyer EL, Brigham KL, Snapper JR. Circulating granulocytes are not essential for lung mechanics changes produced by phorbol myristate acetate. Am Rev Respir Dis 1984; 129:A350 3 Dyer EL, Snapper JR. The role of circulating granulocytes ...

Hereditary hemorrhagic telangiectasia (HHT), also known by the eponym Osler-Weber-Rendu syndrome, is a group of related disorders inherited in an autosomal dominant fashion and characterized by the development of arteriovenous malformations (AVM) in the skin, mucous membranes, and/or internal organs such as brain, lungs, and liver. Its prevalence is currently estimated at one in 5,000 to 8,000....

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%...

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant blood vessel disorder characterized by the presence of arteriovenous malformations (AVMs), epistaxis, and mucocutaneous telangiectases. AVMs are present in lungs, brain, liver, and spine. Children and adults share the same manifestations, with epistaxis and skin telangiectases being the most common. Parents often seek medical ...

Osler-Weber-Rendu disease (OWRD) or Hereditary Hemorrhagic Telangiectasia (HHT) is a rare autosomal dominant disorder that causes muco-cutanesous and visceral vascular dysplasia and results in increased tendency for bleeding [1-4]. Patients with HHT may present with variety of symptoms and management differs accordingly. Epistaxis is the most common symptom of HHT and mucocutaneous telangiectas...