Anti-angiogenic therapeutic strategies in hereditary hemorrhagic telangiectasia

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Anti-angiogenic therapeutic strategies in hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplastic disorder, characterized by recurrent nosebleeds (epistaxis), multiple telangiectases and arteriovenous malformations (AVMs) in major organs. Mutations in Endoglin (ENG or CD105) and Activin receptor-like kinase 1 (ACVRL1 or ALK1) genes of the TGF-β superfamily receptors are responsible for HHT1 and HHT2 res...

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Circulating Angiogenic Cell Dysfunction in Patients with Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder. Circulating angiogenic cells (CACs) play an important role in vascular repair and regeneration. This study was designed to examine the function of CACs derived from patients with HHT. Peripheral blood mononuclear cells (PBMNCs) isolated from patients with HHT and age- and gender-matched healthy volunteers we...

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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....

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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...

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Paradoxical systemic embolization in hereditary hemorrhagic telangiectasia.

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...

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ژورنال

عنوان ژورنال: Frontiers in Genetics

سال: 2015

ISSN: 1664-8021

DOI: 10.3389/fgene.2015.00035