The Lung in Hereditary Hemorrhagic Telangiectasia

被引：47
|
作者：
Dupuis-Girod, Sophie [1 ,2 ]
Cottin, Vincent [3 ,4 ]
Shovlin, C. L. [5 ]
机构：
[1] Hosp Civils Lyon, Hop Femme Mere Enfants, Ctr Reference Natl Malad Rendu Osler, Serv Genet, Bron, France
[2] Univ Lyon 1, Fac Med, Villeurbanne, France
[3] Hosp Civils Lyon, Hop Louis Pradel, Ctr Reference Natl Malad Pulm Rares, Serv Pneumol, Lyon, France
[4] Univ Lyon 1, INRA, UMR754, Lyon, France
[5] Imperial Coll London, Imperial Ctr Translat & Expt Med, NHLI Cardiovasc Sci, London, England
来源：
RESPIRATION | 2017年 / 94卷 / 04期
关键词：
Hereditary hemorrhagic telangiectasia; Pulmonary arteriovenous malformations; Pulmonary hypertension; Anemia; Rare vascular disease; PULMONARY ARTERIOVENOUS-MALFORMATIONS; TO-LEFT SHUNT; TRANSTHORACIC CONTRAST ECHOCARDIOGRAPHY; HEPATIC VASCULAR MALFORMATIONS; RENDU-OSLER-DISEASE; LIVER-TRANSPLANTATION; HEART-FAILURE; ARTERIAL-HYPERTENSION; ENDOTHELIAL-CELLS; DENTAL PROCEDURES;
D O I：
10.1159/000479632
中图分类号：
R56 [呼吸系及胸部疾病];
学科分类号：
摘要：
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 Curacao 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. (C) 2017 S. Karger AG, Basel
引用
收藏
页码：315 / 330
页数：16
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