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Nutritional status, swallowing disorders and respiratory prognosis in adult Duchenne muscular dystrophy patients
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  • Abdallah Fayssoil,
  • Cendrine CHAFFAUT,
  • Helene PRIGENT,
  • Pascal LAFORET,
  • Bernard CLAIR,
  • David ORLIKOWSKI,
  • Adam OGNA,
  • Sylvie CHEVRET,
  • Paris MENG,
  • Djillali ANNANE,
  • Frédéric LOFASO,
  • Pascal CRENN
Abdallah Fayssoil
Hôpital Raymond-Poincare
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Cendrine CHAFFAUT
Hopital Saint-Louis
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Helene PRIGENT
Hopital Raymond-Poincare
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Pascal LAFORET
Hopital Raymond-Poincare
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Bernard CLAIR
Hopital Raymond-Poincare
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David ORLIKOWSKI
Hopital Raymond-Poincare
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Adam OGNA
Hopital Raymond-Poincare
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Sylvie CHEVRET
Hopital Saint-Louis
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Paris MENG
Hopital Raymond-Poincare
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Djillali ANNANE
Hopital Raymond-Poincare
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Frédéric LOFASO
Hopital Raymond-Poincare
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Pascal CRENN
Hopital Raymond-Poincare
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Abstract

Malnutrition and swallowing disorders are common in Duchenne muscular dystrophy (DMD) patients. We assessed, in adult DMD with HMV (home mechanical ventilation) and cough assist device, its prevalence and the relationships with respiratory muscle strength and long-term respiratory prognosis. We reviewed the patients (n=117, age 18 to 39 years (median 24)), followed in a reference center, from 2006 to 2015, to obtain clinical baseline, nutritional status, vital capacity (VC), maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP). Median body mass index was low (15.6). Prevalence of malnutrition, swallowing disorders and gastrostomy were respectively 62, 34 and 11%. BMI and serum albumin level were significantly associated with MIP, MEP and VC. The 1/5-years cumulative incidences of respiratory events (pulmonary sepsis and acute respiratory distress) were respectively 20.7/44.5%. Using univariate analysis, predictive factors for respiratory events were swallowing disorders (p=0.001), transthyretinemia (p=0.034), MIP (p=0.039) and MEP (p=0.03) but not BMI or albuminemia. Using multivariate analysis, only swallowing disorders remained significantly associated with respiratory events (OR=4.2, IC95% 1.31-12.2, p=0.01). If prevalence of malnutrition and swallowing disorders are high in adult DMD on HMV, and respiratory muscles strength associated with serum albumin level and BMI, respiratory prognosis is mainly related to swallowing disorders.

Peer review status:ACCEPTED

22 Oct 2020Submitted to Pediatric Pulmonology
23 Oct 2020Submission Checks Completed
23 Oct 2020Assigned to Editor
25 Oct 2020Reviewer(s) Assigned
25 Nov 2020Review(s) Completed, Editorial Evaluation Pending
29 Nov 2020Editorial Decision: Revise Major
16 Mar 20211st Revision Received
17 Mar 2021Reviewer(s) Assigned
17 Mar 2021Submission Checks Completed
17 Mar 2021Assigned to Editor
11 Apr 2021Review(s) Completed, Editorial Evaluation Pending
12 Apr 2021Editorial Decision: Accept