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<title lang=en>Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases</title>
<creator>Schmitt, Pierre</creator>
<creator>Dalar, Levent</creator>
<creator>Jouneau, Stéphane</creator>
<creator>Toublanc, Bénédicte</creator>
<creator>Camuset, Juliette</creator>
<creator>Chatte, Gérard</creator>
<creator>Cellerin, Laurent</creator>
<creator>Dutau, Hervé</creator>
<creator>Sanchez, Stéphane</creator>
<creator>Sauvage, Maxime</creator>
<creator>Vergnon, Jean-Michel</creator>
<creator>Dury, Sandra</creator>
<creator>Deslée, Gaetan</creator>
<creator>Lebargy, François</creator>
<contributor>Hôpital Maison Blanche</contributor>
<contributor>Institut de recherche, santé, environnement et travail [Rennes] (Irset) ; Université d'Angers (UA) - Université des Antilles et de la Guyane (UAG) - Université de Rennes 1 (UR1) - École des Hautes Études en Santé Publique [EHESP] (EHESP) - Institut National de la Santé et de la Recherche Médicale (INSERM) - Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )</contributor>
<contributor>Centre d'Investigation Clinique [Rennes] (CIC) ; Université de Rennes 1 (UR1) - Hôpital Pontchaillou - Institut National de la Santé et de la Recherche Médicale (INSERM)</contributor>
<contributor>CHU Pontchaillou [Rennes]</contributor>
<contributor>Service d’Oncologie Médicale Thoracique et Digestive ; CHU Nantes</contributor>
<contributor>CHU Reims</contributor>
<contributor>Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne)</contributor>
<contributor>Plasticité de l'épithélium respiratoire dans les conditions normales et pathologiques - UMR-S 903 (PERPMP) ; Université de Reims Champagne-Ardenne (URCA) - Institut National de la Santé et de la Recherche Médicale (INSERM) - SFR CAP Santé (Champagne-Ardenne Picardie Santé) ; Université de Reims Champagne-Ardenne (URCA) - Université de Picardie Jules Verne (UPJV) - Université de Reims Champagne-Ardenne (URCA) - Université de Picardie Jules Verne (UPJV) - CHU de Reims - Hôpital Maison Blanche</contributor>
<contributor>Hôpital Maison Blanche ; CHU Reims</contributor>
<contributor>Unité d'Aide Méthodologique ; Hôpital Robert Debré - CHU Reims</contributor>
<description>International audience</description>
<source>Respiration</source>
<identifier>hal-01331182</identifier>
<identifier>https://hal-univ-rennes1.archives-ouvertes.fr/hal-01331182</identifier>
<source>https://hal-univ-rennes1.archives-ouvertes.fr/hal-01331182</source>
<source>Respiration, 2016, 91 (4), pp.281--287. 〈10.1159/000445029〉</source>
<identifier>DOI : 10.1159/000445029</identifier>
<relation>info:eu-repo/semantics/altIdentifier/doi/10.1159/000445029</relation>
<identifier>PUBMED : 27022925</identifier>
<relation>info:eu-repo/semantics/altIdentifier/pmid/27022925</relation>
<language>en</language>
<subject>[SDV] Life Sciences [q-bio]</subject>
<type>info:eu-repo/semantics/article</type>
<type>Journal articles</type>
<description lang=en>Background: Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by enlargement of the trachea and main bronchi and associated with recurrent respiratory tract infections. Objective: This multicenter, retrospective study was carried out to describe respiratory conditions associated with tracheobronchomegaly. Methods: Nine institutions involved in the ‘Groupe d'Endoscopie de Langue Française' (GELF) participated in this study. A standard form was used to record patient characteristics, treatments and follow-up from medical charts. Results: Seventeen patients, 53% male, aged 58 ± 18 years at diagnosis were included. Recurrent infections revealed MKS in 88% of cases. Main comorbid conditions were diffuse bronchiectasis in 88% of patients and tracheobronchomalacia in 67% of cases. The exacerbation rate was 1.5 exacerbations/patient/year. The main non-respiratory morbid condition was gastroesophageal reflux disease in 29% of cases. Interventional bronchoscopy was performed in seven patients (41%), consisting of laser (n = 2) and tracheal stenting (n = 5). Complications related to stents were observed in 80% of cases with a mean stent duration of 8 months. Four deaths, including three due to respiratory causes, occurred during follow-up. Conclusions: This is the largest series of MKS reported in the literature, showing that bronchiectasis and tracheobronchomalacia are the main associated morbid conditions that constitute a challenge for treatment.</description>
<date>2016-03</date>
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