Titre :
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Assessment of home noninvasive ventilation in myotonic dystrophy type 1 (poster)
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Revue :
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IXèmes journées annuelles de la Société Française de Myologie (SFM), poster T8
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Auteurs :
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IXèmes Journées Annuelles de la Société française de Myologie (SFM) (3-5 novembre 2011; Angers) ;
Hamon MA ;
Meslier N ;
Richard I ;
Aube AC ;
Havet V ;
Person C ;
Dubas F ;
Gagnadoux F ;
Pénisson-Besnier I
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Type de document :
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Article
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Année de publication :
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2011
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Pages :
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p. 36
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Langues:
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Anglais
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Mots-clés :
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colloque
;
dystrophie myotonique de type 1
;
étude de cohorte
;
mouvement respiratoire
;
perspective de recherche
;
prise en charge à domicile
;
qualité de vie
;
somnolence diurne
;
suivi médical
;
trouble du sommeil
;
ventilation non invasive
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Résumé :
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INTRODUCTION : Respiratory problems represent the leading cause of morbidity/mortality in myotonic dystrophy type 1 (DM1). The pertinence of usual criteria for considering noninvasive ventilation (NIV) in neuromuscular diseases is not established in DM1 and NIV compliance and efficacy have been poorly investigated in these patients. AIM : To assess the long term compliance and efficacy of home NIV in DM1 outpatients. METHOD : Among 64 consecutive adult DM1 patients, we retrospectively analyzed data from 24 patients in whom NIV had been prescribed. Nineteen patients had a severe sleep disordered breathing (SDB). Four had a mild-to-moderate SDB associated with excessive daytime sleepiness (n = 3) or PaCO2 > 50 mmHg (n = 1). One patient with severe sleepiness and daytime hypoventilation was started on NIV before the nocturnal recording could be performed. RESULTS : After a mean follow-up of 24 months, 13 (54 %) patients achieved a NIV compliance ? 3h / day (mean: 7.0 ± 2.3 h). Three patients had immediately refused NIV, 1 was not installed at home because of nocturnal worsening on NIV, 7 had abandoned NIV after a mean follow-up of 18 months (range 4 - 40 months). No predictive factor for compliance with NIV was identified. Mean patient satisfaction score was 7.2/10, in contrast with the absence of significant improvement of daytime PaCO2 and Epworth score. CONCLUSION : Our study showed a poor compliance with little benefit in DM1 patients in stable respiratory state considered for NIV. Further studies are required for a better definition of NIV criteria and evaluation in this population.
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Voir aussi :
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