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Feeding difficulties in children and adolescents with spinal muscular atrophy type 2

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dc.contributor.author Wadman, Renske I.
dc.contributor.author de Amicis, Ramona
dc.contributor.author Battezzati, Alberto
dc.contributor.author Bertoli, Simona
dc.contributor.author Davis, Tracey
dc.contributor.author Main, Marion
dc.contributor.author Manzur, Adnan
dc.contributor.author Mastella, Chiara
dc.contributor.author Munot, Pinki
dc.contributor.author Imbrigiotta, Nadia
dc.contributor.author Schottlaender, Lucía V.
dc.contributor.author Sarkozy, Anna
dc.contributor.author Trucco, Federica
dc.contributor.author Baranello, Giovanni
dc.contributor.author Scoto, Mariacristina
dc.contributor.author Muntoni, Francesco
dc.contributor.author Brusa, Chiara
dc.date.accessioned 2021-05-17T14:00:23Z
dc.date.available 2021-05-17T14:00:23Z
dc.date.issued 2020-12-19
dc.identifier.citation Wadman RI, De Amicis R, Brusa C, Battezzati A, Bertoli S, Davis T, Main M, Manzur A, Mastella C, Munot P, Imbrigiotta N, Schottlaender L, Sarkozy A, Trucco F, Baranello G, Scoto M, Muntoni F. Feeding difficulties in children and adolescents with spinal muscular atrophy type 2. Neuromuscul Disord. 2020 Dec 19:S0960-8966(20)30699-4. doi: 10.1016/j.nmd.2020.12.007. Epub ahead of print. es_ES
dc.identifier.uri https://repositorio.fleni.org.ar/xmlui/handle/123456789/484
dc.identifier.uri https://doi.org/10.1016/j.nmd.2020.12.007
dc.description.abstract Disease course of feeding difficulties in spinal muscular atrophy type 2 is not well documented. Disease-modifying therapies rapidly change the trajectory of motor function and survival in spinal muscular atrophy, but effects on co-morbidities like bulbar function are unknown. We analysed data concerning feeding problems and their standard of care treatment in 146 patients with spinal muscular atrophy type 2. Data were collected from two separate cohorts: one single-centre retrospective chart review study from the United Kingdom (London), and one prospective questionnaire-based multicentre study from Italy. Cumulatively feeding difficulties were present in 88 patients (60%) in these 2 cohorts. Median age at onset of problems was 6.5years (range 0-16.5 years). Eighty-two patients (60%) showed periods of underweight according to age adjusted body mass index, and thirty-six patients (25%) showed malnourishment with a significant drop on their weight curves. Enteral feeding was indicated in 23 out of 72 patients in the UK cohort (32%) because of weight loss, oropharyngeal dysphagia or aspiration. Gastrostomy and its placement was generally well tolerated, uncomplicated in 96%, never reversed and performed without Nissen fundoplication in 66% of patients. After gastrostomy chest infections improved in 80% and nutritional status (e.g., Body Mass Index) in 84% of patients. These results show that feeding difficulties are a common problem in spinal muscular atrophy type 2. Treatment strategies should be tailor-made on the symptoms and needs of the individual patient. es_ES
dc.language.iso eng es_ES
dc.publisher Pergamon Press es_ES
dc.rights info:eu-repo/semantics/openAccess
dc.rights.uri https://creativecommons.org/licenses/by/2.5/ar/
dc.subject Thinness es_ES
dc.subject Delgadez es_ES
dc.subject Muscular Atrophy es_ES
dc.subject Atrofia Muscular es_ES
dc.title Feeding difficulties in children and adolescents with spinal muscular atrophy type 2 es_ES
dc.type info:eu-repo/semantics/article es_ES
dc.type info:eu-repo/semantics/publishedVersion
dc.description.fil Fil: Schottlaender, Lucía V. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Fleni. Departamento de Neurología; Argentina. University College London. Great Ormond Street Institute of Child Health. NIHR Great Ormond Street Hospital Biomedical Research Centre. Dubowitz Neuromuscular Centre; Reino Unido. Great Ormond Street Hospital Trust; Reino Unido. University College London. Institute of Neurology. Department of Molecular Neuroscience; Reino Unido.
dc.description.fil Fil: Wadman, Renske I. University College London. Great Ormond Street Institute of Child Health. NIHR Great Ormond Street Hospital Biomedical Research Centre. Dubowitz Neuromuscular Centre; Reino Unido. Great Ormond Street Hospital Trust; Reino Unido. University Medical Center Utrecht. UMC Utrecht Brain Center. Department of Neurology and Neurosurgery; Países Bajos.
dc.description.fil Fil: De Amicis, Ramona. University of Milan. Department of Food Environmental and Nutritional Sciences. International Center for the Assessment of Nutritional Status; Italia.
dc.description.fil Fil: Battezzati, Alberto. University of Milan. Department of Food Environmental and Nutritional Sciences. International Center for the Assessment of Nutritional Status; Italia.
dc.description.fil Fil: Brusa, Chiara. University College London. Great Ormond Street Institute of Child Health. NIHR Great Ormond Street Hospital Biomedical Research Centre. Dubowitz Neuromuscular Centre; Reino Unido. Great Ormond Street Hospital Trust; Reino Unido.
dc.description.fil Fil: Bertoli, Simona. University of Milan. Department of Food Environmental and Nutritional Sciences. International Center for the Assessment of Nutritional Status; Italia. IRCCS Istituto Auxologico Italiano. Department of Endocrine and Metabolic Diseases. Obesity Unit and Laboratory of Nutrition and Obesity Research; Italia.
dc.description.fil Fil: Davis, Tracey. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido.
dc.description.fil Fil: Main, Marion. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido.
dc.description.fil Fil: Manzur, Adnan. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido.
dc.description.fil Fil: Mastella, Chiara. Mangiagalli e Regina Elena Hospital. Early Habilitation Service; Italia.
dc.description.fil Fil: Munot, Pinki. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido.
dc.description.fil Fil: Imbrigiotta, Nadia. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido.
dc.description.fil Fil: Sarkozy, Anna. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido.
dc.description.fil Fil: Trucco, Federica. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido.
dc.description.fil Fil: Baranello, Giovanni. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido. Ormond Street Hospital Trust; Reino Unido. Fondazione IRCCS Istituto Neurologico Carlo Besta. Developmental Neurology Unit; Italia.
dc.description.fil Fil: Scoto, Mariacristina. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido.
dc.description.fil Fil: Muntoni, Francesco. Great Ormond Street Hospital for Children. Dubowitz Neuromuscular Centre; Reino Unido.
dc.relation.ispartofCOUNTRY Inglaterra
dc.relation.ispartofCITY Oxford
dc.relation.ispartofTITLE Neuromuscular disorders : NMD.
dc.relation.ispartofISSN 1873-2364
dc.type.snrd info:ar-repo/semantics/artículo es_ES


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