The Lung in Hereditary Hemorrhagic Telangiectasia | Zendy

Sophie DupuisGirod | Zendy; Vincent Cottin | Zendy; Claire L. Shovlin | Zendy

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The Lung in Hereditary Hemorrhagic Telangiectasia

Author(s) -

Sophie DupuisGirod,

Vincent Cottin,

Claire L. Shovlin

Publication year - 2017

Publication title -

respiration

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.264

H-Index - 81

eISSN - 1423-0356

pISSN - 0025-7931

DOI - 10.1159/000479632

Subject(s) - medicine , telangiectasia , lung , gastrointestinal tract , pathology , genetic disorder , disease

Hereditary hemorrhagic telangiectasia (HHT) is a dominantly inherited genetic vascular disorder with an estimated prevalence of 1 in 6,000, characterized by recurrent epistaxis, cutaneous telangiectasia, and arteriovenous malformations (AVMs) that affect many organs including the lungs, gastrointestinal tract, liver, and brain. Its diagnosis is based on the Curaçao criteria, and is considered definite if at least 3 of the 4 following criteria are fulfilled: (1) spontaneous and recurrent epistaxis, (2) telangiectasia, (3) a family history, and (4) pulmonary, liver, cerebral, spinal, or gastrointestinal AVMs. The focus of this review is on delineating how HHT affects the lung.

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