title: Causes of death in French cystic fibrosis patients: The need for improvement in transplantation referral strategies!
abstract: BACKGROUND
  Little data exist on causes of death in cystic fibrosis (CF) patients in the era of lung transplantation.
  
  
  METHODS
  Deaths in CF patients in France (2007-2010) were identified using the French CF Registry and causes of deaths were determined based on medical files by a mortality adjudication committee.
  
  
  RESULTS
  Of 256 deaths, half occurred after lung transplantation and were related to early or late complications of transplantation, whereas half occurred in patients who did not receive lung transplantation and were primarily related to respiratory failure or massive hemoptysis. Among patients who did not receive lung transplantation, only 19% died while waiting on a lung transplantation list. Lack of listing for lung transplantation was primarily related to late, or to lack of transplantation referral, rather than to contraindication to transplantation.
  
  
  CONCLUSIONS
  These data suggest that improvement in transplantation referral strategies may result in transplantation-related survival benefits.
authors.affiliation: Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: clemence.martin@aphp.fr.
authors.forename: Clémence
authors.initials: C
authors.lastname: Martin
authors.affiliation: Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: cecileweiline@gmail.com.
authors.forename: Cécile
authors.initials: C
authors.lastname: Hamard
authors.affiliation: Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: reem.kanaan@aphp.fr.
authors.forename: Reem
authors.initials: R
authors.lastname: Kanaan
authors.affiliation: Université Paris Descartes, Sorbonne Paris Cité, Paris, France; Department of Cardiac Surgery, Hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux de Paris, Paris, France. Electronic address: veronique.boussaud@aphp.fr.
authors.forename: Véronique
authors.initials: V
authors.lastname: Boussaud
authors.affiliation: Department of Respiratory Medicine, Hôpital Foch, Suresnes, France. Electronic address: d.grenet@hopital-foch.org.
authors.forename: Dominique
authors.initials: D
authors.lastname: Grenet
authors.affiliation: Department of Pediatrics A, American Memorial Hospital, Reims, France. Electronic address: mabely@chu-reims.fr.
authors.forename: Michel
authors.initials: M
authors.lastname: Abély
authors.affiliation: Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: dominique.hubert@aphp.fr.
authors.forename: Dominique
authors.initials: D
authors.lastname: Hubert
authors.affiliation: Department of Pediatric Gastroenterology and Respiratory Medicine, Hôpital Robert Debré, Assistance Publique Hôpitaux de Paris, University Paris 7, Paris, France. Electronic address: anne.munck@aphp.fr.
authors.forename: Anne
authors.initials: A
authors.lastname: Munck
authors.affiliation: Vaincre la Mucoviscidose, Paris, France. Electronic address: llemonnier@vaincrelamuco.org.
authors.forename: Lydie
authors.initials: L
authors.lastname: Lemonnier
authors.affiliation: Department of Respiratory Medicine, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: pierre-regis.burgel@aphp.fr.
authors.forename: Pierre-Régis
authors.initials: PR
authors.lastname: Burgel
mesh_terms: D000328:Adult; D002423:Cause of Death / Q000639:trends; D003550:Cystic Fibrosis / Q000150:complications / Q000401:mortality* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D005602:France / Q000453:epidemiology; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D011379:Prognosis; D012017:Referral and Consultation / Q000592:standards*; D012042:Registries; D012131:Respiratory Insufficiency / Q000209:etiology / Q000401:mortality* / Q000601:surgery; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D055815:Young Adult
keywords: Cystic fibrosis exacerbation; Hemoptysis; Lung transplantation; Respiratory failure