Nutritional Challenges in Duchenne Muscular Dystrophy
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作者:
Salera, Simona
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Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, ItalyUniv Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, Italy
Salera, Simona
[1
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Menni, Francesca
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Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, ItalyUniv Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, Italy
Menni, Francesca
[1
]
Moggio, Maurizio
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机构:
Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Neurosci, Neuromuscular & Rare Dis Unit, I-20122 Milan, ItalyUniv Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, Italy
Moggio, Maurizio
[2
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Guez, Sophie
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Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, ItalyUniv Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, Italy
Guez, Sophie
[1
]
Sciacco, Monica
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Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Neurosci, Neuromuscular & Rare Dis Unit, I-20122 Milan, ItalyUniv Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, Italy
Sciacco, Monica
[2
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Esposito, Susanna
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Univ Perugia, Pediat Clin, I-06129 Perugia, ItalyUniv Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, Italy
Esposito, Susanna
[3
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机构:
[1] Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Pathophysiol & Transplantat, Pediat Highly Intens Care Unit, I-20122 Milan, Italy
[2] Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Neurosci, Neuromuscular & Rare Dis Unit, I-20122 Milan, Italy
Neuromuscular diseases (NMDs) represent a heterogeneous group of acquired or inherited conditions. Nutritional complications are frequent in NMDs, but they are sometimes underestimated. With the prolongation of survival in patients with NMDs, there are several nutritional aspects that are important to consider, including the deleterious effects of overnutrition on glucose metabolism, mobility, and respiratory and cardiologic functions; the impact of hyponutrition on muscle and ventilatory function; constipation and other gastrointestinal complications; chewing/swallowing difficulties with an increased risk of aspiration that predisposes to infectious diseases and respiratory complications; as well as osteoporosis with an associated increased risk of fractures. The aim of this review is to provide a comprehensive analysis of the nutritional aspects and complications that can start in children with Duchenne muscular dystrophy (DMD) and increase with ageing. These aspects should be considered in the transition from paediatric clinics to adult services. It is shown that appropriate nutritional care can help to improve the quality of life of DMD patients, and a multidisciplinary team is needed to support nutrition challenges in DMD patients. However, studies on the prevalence of overnutrition and undernutrition, gastrointestinal complications, infectious diseases, dysphagia, and reduced bone mass in the different types of NMDs are needed, and appropriate percentiles of weight, height, body mass index, and body composition appear to be extremely important to improve the management of patients with NMD.
机构:
Univ Michigan, Sch Med, Dept Human Genet, M4708 Med Sci 2,Box 0618, Ann Arbor, MI 48190 USAUniv Michigan, Sch Med, Dept Human Genet, M4708 Med Sci 2,Box 0618, Ann Arbor, MI 48190 USA
机构:
Hosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, SpainHosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Gomez, J. Armijo
Liz, M.
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Hosp St Joan Deu, Dept Neurol, Epilepsy Unit, Barcelona, SpainHosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Liz, M.
Ortez, C.
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机构:
Hosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Inst Recerca St Joan Deu, Appl Res Neuromuscular Dis, Barcelona, Spain
ISCIII, Ctr Biomed Res Network Rare Dis CIBERER, Barcelona, SpainHosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Ortez, C.
Dominguez-Carral, J.
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Hosp St Joan Deu, Dept Neurol, Epilepsy Unit, Barcelona, SpainHosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Dominguez-Carral, J.
Exposito-Escudero, J.
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机构:
Hosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Inst Recerca St Joan Deu, Appl Res Neuromuscular Dis, Barcelona, SpainHosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Exposito-Escudero, J.
Carrera-Garcia, L.
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机构:
Hosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Inst Recerca St Joan Deu, Appl Res Neuromuscular Dis, Barcelona, SpainHosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Carrera-Garcia, L.
Natera De Benito, D.
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机构:
Hosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Inst Recerca St Joan Deu, Appl Res Neuromuscular Dis, Barcelona, SpainHosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Natera De Benito, D.
Nascimento, A.
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机构:
Hosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain
Inst Recerca St Joan Deu, Appl Res Neuromuscular Dis, Barcelona, Spain
ISCIII, Ctr Biomed Res Network Rare Dis CIBERER, Barcelona, SpainHosp St Joan Deu, Dept Neurol, Neuromuscular Unit, Barcelona, Spain