Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases
| العنوان: | Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases |
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| المؤلفون: | Hervé Dutau, François Lebargy, Levent Dalar, Bénédicte Toublanc, Stéphane Sanchez, Pierre Schmitt, Gérard Chatte, Sandra Dury, Juliette Camuset, Jean-Michel Vergnon, Laurent Cellerin, Gaëtan Deslée, Maxime Sauvage, Stéphane Jouneau |
| المساهمون: | Hôpital Maison Blanche, Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-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 ), Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], Service d’Oncologie Médicale Thoracique et Digestive, Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Universitaire de Reims (CHU Reims), Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Plasticité de l'épithélium respiratoire dans les conditions normales et pathologiques - UMR-S 903 (PERPMP), 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)-Centre Hospitalier Universitaire de Reims (CHU Reims)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Reims Champagne-Ardenne (URCA), Unité d'Aide Méthodologique, Hôpital Robert Debré-Centre Hospitalier Universitaire de Reims (CHU Reims), Université d'Angers (UA)-Université de Rennes (UR)-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 ), Université de Rennes (UR)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Université de Reims Champagne-Ardenne (URCA)-Centre Hospitalier Universitaire de Reims (CHU Reims)-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 Reims Champagne-Ardenne (URCA) |
| المصدر: | Respiration Respiration, Karger, 2016, 91 (4), pp.281--287. ⟨10.1159/000445029⟩ Respiration, 2016, 91 (4), pp.281--287. ⟨10.1159/000445029⟩ |
| بيانات النشر: | HAL CCSD, 2016. |
| سنة النشر: | 2016 |
| مصطلحات موضوعية: | Pulmonary and Respiratory Medicine, Tracheobronchomegaly, Adult, Male, Pediatrics, medicine.medical_specialty, Exacerbation, medicine.medical_treatment, [SDV]Life Sciences [q-bio], 03 medical and health sciences, 0302 clinical medicine, Bronchoscopy, medicine, Humans, 030212 general & internal medicine, Respiratory system, Age of Onset, Respiratory Tract Infections, Aged, Retrospective Studies, Tracheobronchomalacia, Bronchiectasis, business.industry, Reflux, Stent, Retrospective cohort study, Middle Aged, medicine.disease, 3. Good health, 030228 respiratory system, Pulmonary Emphysema, Gastroesophageal Reflux, Female, business, Tomography, X-Ray Computed |
| الوصف: | 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. |
| اللغة: | English |
| تدمد: | 0025-7931 1423-0356 |
| DOI: | 10.1159/000445029⟩ |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3124f9577e688a0e8c42efcee9acf8e7 https://hal-univ-rennes1.archives-ouvertes.fr/hal-01331182 |
| Rights: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....3124f9577e688a0e8c42efcee9acf8e7 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 00257931 14230356 |
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| DOI: | 10.1159/000445029⟩ |