Nutritional status, swallowing disorders, and respiratory prognosis in adult Duchenne muscular dystrophy patients.

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Abstrakt

Tytuł:: Nutritional status, swallowing disorders, and respiratory prognosis in adult Duchenne muscular dystrophy patients.
Autorzy:: Fayssoil A; Service de Médecine intensive et Réanimation/Unité de ventilation à domicile, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; CRMR des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Service de cardiologie, Centre de référence des cardiomyopathies et des troubles du rythme cardiaque héréditaires ou rares, Hôpital Ambroise Paré, AP-HP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Boulogne-Billancourt, France.; Centre d'Investigation clinique (CIC) 1429 INSERM, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Chaffaut C; Service de Biostatistiques et Information Médicale (SBIM), Hôpital Saint Louis, APHP, Université Paris, Paris, France.
Prigent H; CRMR des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Centre d'Investigation clinique (CIC) 1429 INSERM, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Service de Physiologie-Explorations fonctionnelles, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Laforet P; CRMR des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Centre d'Investigation clinique (CIC) 1429 INSERM, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Service de Neurologie/Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Clair B; Service de Médecine intensive et Réanimation/Unité de ventilation à domicile, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; CRMR des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Orlikowski D; Service de Médecine intensive et Réanimation/Unité de ventilation à domicile, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; CRMR des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Centre d'Investigation clinique (CIC) 1429 INSERM, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Ogna A; Service de Médecine intensive et Réanimation/Unité de ventilation à domicile, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Chevret S; Service de Biostatistiques et Information Médicale (SBIM), Hôpital Saint Louis, APHP, Université Paris, Paris, France.
Meng P; Service de Médecine intensive et Réanimation/Unité de ventilation à domicile, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Annane D; Service de Médecine intensive et Réanimation/Unité de ventilation à domicile, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; CRMR des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Centre d'Investigation clinique (CIC) 1429 INSERM, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Lofaso F; CRMR des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Centre d'Investigation clinique (CIC) 1429 INSERM, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Service de Physiologie-Explorations fonctionnelles, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Crenn P; CRMR des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Centre d'Investigation clinique (CIC) 1429 INSERM, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.; Unité de Nutrition clinique transversale, Hôpital Raymond Poincaré, APHP Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.
Źródło:: Pediatric pulmonology [Pediatr Pulmonol] 2021 Jul; Vol. 56 (7), pp. 2146-2154. Date of Electronic Publication: 2021 May 03.
Typ publikacji:: Journal Article
Język:: English
Imprint Name(s):: Publication: <2005-> : Hoboken, NJ : Wiley-Liss
Original Publication: [Philadelphia, PA] : W.B. Saunders, [c1985-
MeSH Terms:: Deglutition Disorders*/epidemiology
Deglutition Disorders*/etiology
Muscular Dystrophy, Duchenne*/complications
Adolescent ; Adult ; Deglutition ; Humans ; Nutritional Status ; Prognosis ; Respiratory Muscles ; Young Adult
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Contributed Indexing:: Keywords: Duchenne muscular dystrophy; albumin; home mechanical ventilation; malnutrition; prognosis
Entry Date(s):: Date Created: 20210503 Date Completed: 20211124 Latest Revision: 20211124
Update Code:: 20240104
DOI:: 10.1002/ppul.25430
PMID:: 33939888
: Czasopismo naukowe

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Malnutrition and swallowing disorders are common in Duchenne muscular dystrophy (DMD) patients. We assessed, in adult DMD with home mechanical ventilation (HMV) 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 18-39 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), and maximal expiratory pressure (MEP). The median body mass index (BMI) was low (15.6 kg/m²). Included patients had severe restrictive respiratory function with a median VC of 10.5% [7-17] of the predicted value. All patients were on HMV. 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-year/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 = .001), transthyretinemia (p = .034), MIP (p = .039), and MEP (p = .03) but not BMI or albuminemia. Using multivariate analysis, only swallowing disorders remained significantly associated with respiratory events (OR = 4.2, IC 95% 1.31-12.2, p = .01). In conclusion, this study highlights the interrelationships between nutritional intake, swallowing function, airway clearance, and respiratory function in adult DMD. A multidisciplinary approach focusing on these previous factors is essential to optimize DMD patient health.
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