Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu syndrome)

Hereditary hemorrhagic telangiectasia/avastin.

This is the first scientific report of hereditary hemorrhagic telangiectasia (HHT) epistaxis treatment by intranasal spraying of the vascular endothelial growth factor (VEGF) inhibitor bevacizumab (Avastin). Epistaxis in patients with HHT is a morbid, mortal condition that is difficult and unpleasant to manage. Nasal telangiectasia growth is modulated by VEGF, which is elevated in HHT patients....

Hereditary Hemorrhagic Telangiectasia Management.

Hereditary hemorrhagic telangiectasia (HHT) is an inherited disorder that can lead to frequent and severe sequelae. Although the condition has no cure, many of its physical symptoms can be managed to improve the quality of life and lower the risk of life-threatening complications. This article discusses HHT presentation, genetic screening, diagnosis, and management. The management of HHT can in...

Platypnea-orthodeoxia syndrome as a presentation of hereditary hemorrhagic telangiectasia.

A 57-year-old white woman presented with a 3-month history of progressive shortness of breath. Although she attributed her initial subtle symptoms of mild dyspnea as a result of exertion during exercise to “getting older,” she sought evaluation 1 month before presentation because of the onset of a cough productive of purulent sputum. A chest radiograph demonstrated a consolidation in the right ...

Cerebrovascular Manifestations of Hereditary Hemorrhagic Telangiectasia.

Hereditary Hemorrhagic Telangiectasia with Hepatic Vascular Malformations

Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant hereditary disease. Early diagnosis is important to avoid complications from vascular lesions, but diagnosis is difficult in asymptomatic patients. A 69-year-old Japanese male patient was referred to our hospital for evaluation of hepatic vascular malformations. He had mild anemia with iron deficiency, and dynamic contrast...