Effect and impact of mechanical ventilation in myotonic dystrophy type 1: a prospective cohort study.

Autor:	Boussaïd G; CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, Garches, France.; Association Française contre les Myopathies-Téléthon, Évry, France.; Université de Versailles Saint-Quentin-en-Yvelines, INSERM U1179, Versailles, France., Prigent H; Université de Versailles Saint-Quentin-en-Yvelines, INSERM U1179, Versailles, France.; Service d'Explorations Fonctionnelles Respiratoires, AP-HP, Hôpital Raymond Poincaré, Garches, France., Laforet P; Institut de Myologie, Centre de Référence de Pathologie Neuromusculaire Paris-Est, GH Pitié-Salpêtrière, Paris, France., Raphaël JC; Pôle de ventilation à domicile, AP-HP, Hôpital Raymond Poincaré, Garches, France., Annane D; CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, Garches, France.; Université de Versailles Saint-Quentin-en-Yvelines, INSERM U1179, Versailles, France.; Pôle de ventilation à domicile, AP-HP, Hôpital Raymond Poincaré, Garches, France., Orlikowski D; CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, Garches, France.; Université de Versailles Saint-Quentin-en-Yvelines, INSERM U1179, Versailles, France.; Pôle de ventilation à domicile, AP-HP, Hôpital Raymond Poincaré, Garches, France., Lofaso F; Association Française contre les Myopathies-Téléthon, Évry, France.; Université de Versailles Saint-Quentin-en-Yvelines, INSERM U1179, Versailles, France.; Service d'Explorations Fonctionnelles Respiratoires, AP-HP, Hôpital Raymond Poincaré, Garches, France.
Jazyk:	angličtina
Zdroj:	Thorax [Thorax] 2018 Nov; Vol. 73 (11), pp. 1075-1078. Date of Electronic Publication: 2018 Mar 23.
DOI:	10.1136/thoraxjnl-2017-210610
Abstrakt:	Few studies have assessed the impact of home ventilation in patients with myotonic dystrophy type 1 (DM1) and no specific recommendations are available. We assessed the survival associated with category of home ventilation adherence of patients with DM1 followed up at a home ventilation unit using a Cox proportional hazards model. 218 patients were included; those who refused or delayed their acceptance of non-invasive ventilation were at higher risk for severe events (invasive ventilation or death) (P=0.03). Risk of death was associated with orthopnoea (HR 2.37; 95% CI 1.17 to 4.80; P<0.02) and adherence category (100 to 90% vs >75%: HR 3.26; 95% CI 1.32 to 8.04; P<0.03). Failure to use home ventilation as prescribed may be associated with increased mortality in patients with DM1. Competing Interests: Competing interests: None declared. (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)
Databáze:	MEDLINE
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