Outcomes of Hospitalised Muscular Dystrophy Patients.
BACKGROUND
In France, referral centres in teaching hospitals were created 10 years ago to provide MD patients with treatments and follow-up designed to prevent complications and improve outcomes. Respiratory failure is a major cause of death among subjects with MD, and its prevention and treatment can serve as a touchstone for assessing the effectiveness of MD care pathways.


OBJECTIVE
We report data from a preliminary study of admissions of MD patients in France and of factors associated with mortality, with special emphasis on respiratory failure [RF].


METHODS
Retrospective analysis of the data from the French nationwide hospital database [Programme de M é dicalisation des syst é mes d'information, PMSI] for 2009 and 2010.


RESULTS
7187 admissions of patients with MD were included in the study. Most admissions were to teaching hospitals [5913/7187, 82.3%]. 302 [4.2%] patients were admitted for RF requiring invasive ventilation, 924 [12.9%] for RF requiring only NIV or high-flow oxygen therapy, and 5961 [82.9%] required no respiratory assistance. 494 [6.9%] admissions occurred on an emergency basis. 77/7187 [1.1%] patients died while hospitalised. Teaching-hospital admission was associated with lower frequencies of emergency admission [3.08% vs. 24.5%, p < 0.01] and in-hospital death [0.71% vs. 2.75%, p < 0.01].


CONCLUSIONS
Our data suggest that there is room for improvement in care pathways for MD patients requiring admission. Admission to referral centres may provide the best outcomes.Further studies are needed to assess associations between healthcare pathways and outcomes of MD subjects.
CIC 1429 Inserm, Raymond Poincare Teaching Hospital, AP-HP, Garches, France.|Association Française contre les Myopathies (AFM)-Téléthon, Evry, France.|UMR 1179 End-icap, Inserm, Université de Versailles Saint-Quentin-en-Yvelines, France.
Ghilas
G
Boussaid
Department of Physiology and Functional Testing, Raymond Poincare Teaching Hospital, AP-HP, Garches, France.|UMR 1179 End-icap, Inserm, Université de Versailles Saint-Quentin-en-Yvelines, France.
Hélène
H
Prigent
Hox-Com Analytiques, Vincennes, France.
Gilbert
G
Caranhac
CIC 1429 Inserm, Raymond Poincare Teaching Hospital, AP-HP, Garches, France.
Djillali
D
Annane
Association Française contre les Myopathies (AFM)-Téléthon, Evry, France.
Christian
C
Devaux
CIC 1429 Inserm, Raymond Poincare Teaching Hospital, AP-HP, Garches, France.|UMR 1179 End-icap, Inserm, Université de Versailles Saint-Quentin-en-Yvelines, France.
David
D
Orlikowski
Association Française contre les Myopathies (AFM)-Téléthon, Evry, France.|Department of Physiology and Functional Testing, Raymond Poincare Teaching Hospital, AP-HP, Garches, France.|UMR 1179 End-icap, Inserm, Université de Versailles Saint-Quentin-en-Yvelines, France.
Frédéric
F
Lofaso
D000328:Adult; D004632:Emergency Medical Services; D005260:Female; D005602:France; D017052:Hospital Mortality; D006760:Hospitalization; D006784:Hospitals, Teaching; D006801:Humans; D008297:Male; D009136:Muscular Dystrophies / Q000401:mortality* / Q000628:therapy*; D000074286:Preliminary Data; D012131:Respiratory Insufficiency / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D016896:Treatment Outcome
Muscular dystrophy; artificial/methods; humans; inpatients; outcome assessment (healthcare); respiration; retrospective studies