title: Evolution of life expectancy of patients with Duchenne muscular dystrophy at AFM Yolaine de Kepper centre between 1981 and 2011.
abstract: OBJECTIVE
  Retrospective study over the last 30 years of life expectancy in patients suffering from Duchenne muscular dystrophy (DMD). Analysis of the role of ventilatory assistance and causes of death.
  
  
  METHODS
  One hundred and nineteen adult DMD patients were hosted during 1981 to 2011 at AFM Yolaine de Kepper centre, Saint-Georges-sur-Loire, France. Patients' life expectancy was calculated using Kaplan-Meier model.
  
  
  RESULTS
  Life expectancy without or with ventilatory assistance was 22.16 and 36.23 years, respectively. Similarly, life expectancy of patients born from 1970 (mostly with ventilatory assistance) was 40.95 years old from 1970 and 25.77 years old before 1970. Causes of death changed. Cardiac origins of death have increased from 8% to 44%.
  
  
  CONCLUSIONS
  Ventilator assistance, in this study mostly through tracheotomy prolongs by more than 15 years life expectancy of DMD patients. It allows conservation of a satisfactory quality of life, and should be systematically proposed to patients.
authors.affiliation: Service de MPR neurologique, hôpital Saint-Jacques, CHU de Nantes, 85, rue Saint-Jacques, 44093 Nantes cedex 1, France; Centre AFM Yolaine de Kepper, 49170 Saint-Georges-Sur-Loire, France; Centre de référence des maladies neuromusculaires rares Nantes Angers, CHU de Nantes, 44093 Nantes cedex 1, France. Electronic address: pierre.kieny@orange.fr.
authors.forename: P
authors.initials: P
authors.lastname: Kieny
authors.forename: S
authors.initials: S
authors.lastname: Chollet
authors.forename: P
authors.initials: P
authors.lastname: Delalande
authors.forename: M
authors.initials: M
authors.lastname: Le Fort
authors.forename: A
authors.initials: A
authors.lastname: Magot
authors.forename: Y
authors.initials: Y
authors.lastname: Pereon
authors.forename: B
authors.initials: B
authors.lastname: Perrouin Verbe
mesh_terms: D000293:Adolescent; D000328:Adult; D002423:Cause of Death / Q000639:trends; D006801:Humans; D053208:Kaplan-Meier Estimate; D008017:Life Expectancy / Q000639:trends*; D020388:Muscular Dystrophy, Duchenne / Q000401:mortality* / Q000628:therapy*; D012121:Respiration, Artificial / Q000706:statistics & numerical data*; D012189:Retrospective Studies; D055815:Young Adult
keywords: Assistance ventilatoire; Duchenne muscular dystrophy; Dystrophie musculaire de Duchenne; Espérance de vie; Survival; Tracheotomy; Trachéotomie; Ventilatory assistance