The lung in hereditary hemorrhagic telangiectasia
S Dupuis-Girod, V Cottin, CL Shovlin - Respiration, 2017 - karger.com
S Dupuis-Girod, V Cottin, CL Shovlin
Respiration, 2017•karger.comHereditary 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 …
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 …
Abstract
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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