Nutrição e Distrofia Muscular de Duchenne – Artigo 10
The Relationship between Obesity and Clinical Outcomes in Young People with Duchenne Muscular Dystrophy
Abstract: Background: Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular disorder. Young people withDMDhave high rates of obesity. There is emerging evidence that a higher BMI may negatively affect clinical outcomes in DMD. This study aimed to explore the relationship between obesity and clinical outcomes in DMD.
Methods: This was a retrospective clinical audit of young people (two–21 years) with DMD. Height and weight were collected to calculate BMI z-scores to ...
Nutrição e Distrofia Muscular de Duchenne – Artigo 9
When dysbiosis meets dystrophy: an unwanted gut-muscle connection
Duchenne muscular dystrophy (DMD) is a devastating neuromuscular degenerative disease with no known cure to date. In recent years, the hypothesis of a “gutmuscle axis” has emerged suggesting that bidirectional communication between the gut microbiota and the muscular system regulates the muscular function and may be perturbed in several muscular disorders. In addition, the excessive consumption of sugar and of lipid-rich processed food products are factors that further aggravate the phenotype for such diseases and ...
Nutrição e Distrofia Muscular de Duchenne – Artigo 8
A systematic literature review and meta-analysis of the effectiveness of vitamin D supplementation for patients with Duchenne muscular dystrophy
A b s t r a c t
We conducted a systematic literature review and meta-analysis on the effectiveness of vitamin D supplementation in maintaining or restoring vitamin D levels in Duchenne muscular dystrophy. Due to a lack of randomised controlled trials, cross-sectional and retrospective and prospective cohort studies were taken as the best available evidence. Inclusion criteria included reporting mean serum vitamin D levels in a supplement-taking ...
Nutrição e Distrofia Muscular de Duchenne – Artigo 7
Frequency of Delayed Puberty in Boys with Contemporary Management of Duchenne Muscular Dystrophy
Abstract
Background: Delayed puberty is thought to be common in boys with Duchenne muscular dystrophy (DMD) treated with long term oral glucocorticoid. This study aims to report the frequency of delayed puberty in DMD from examination by a paediatric endocrinologist alongside detailed endocrine investigations.
Methods: All boys with DMD aged at least 14 years in January 2022 known to the paediatric neuromuscular service (2016-2022) were included in this study. Delayed puberty was defined ...
Nutrição e Distrofia Muscular de Duchenne – Artigo 6
Nutritional Issues among Children with Duchenne Muscular Dystrophy—Incidence of Deficiency and Excess Body Mass
Abstract: The progression of Duchenne muscular dystrophy (DMD)requires the assessment of nutritional disturbances at each stage of the disease. The purpose of this study was to assess the nutritional status in various ages of boys with DMD using screening and in-depth evaluation methods. Body composition by Dual X-ray Absorptiometry (DXA), basal metabolic rate (BMR) by indirect calorimetry, a questionnaire of nutritional status—Pediatric Nutrition Screening Tool (PNST)—and ...
Nutrição e Distrofia Muscular de Duchenne – Artigo 5
Duchenne muscular dystrophy
Abstract
Duchenne muscular dystrophy is a severe, progressive, muscle-wasting disease that leads to difficulties with movement and, eventually, to the need for assisted ventilation and premature death. The disease is caused by mutations in DMD (encoding dystrophin) that abolish the production of dystrophin in muscle. Muscles without dystrophin are more sensitive to damage, resulting in progressive loss of muscle tissue and function, in addition to
cardiomyopathy. Recent studies have greatly deepened our understanding of the primary and secondary pathogenetic ...
Nutrição e Distrofia Muscular de Duchenne – Artigo 4
Distrofia muscular de Duchenne: uma revisão narrativa
RESUMO
A Distrofia Muscular de Duchenne (DMD) é uma condição genética progressiva e heterogênea, caracterizada primordialmente pela degradação da força muscular, resultando em incapacidade de locomoção e, eventualmente, comprometimento dos músculos respiratórios e cardíacos. Conhecida pela sua ligação ao cromossomo X, afeta majoritariamente indivíduos do sexo masculino e mulheres com Síndrome de Turner, devido à sua transmissão recessiva ligada ao X. Este estudo tem como objetivo principal definir a Distrofia ...
Nutrição e Distrofia Muscular de Duchenne – Artigo 3
Recomendações nutricionais na Distrofia Muscular de Duchenne
RESUMO
OBJETIVO: Pesquisar as recomendações nutricionais para pacientes com Distrofia Muscular de Duchenne (DMD).
MÉTODO: Trata- se de uma revisão integrativa da literatura, através de levantamento bibliográfico nas bases cientificas PubMed e Scielo, foram utilizadas as palavras chaves: Distrofia Muscular de Duchenne em combinação com os termos Nutrição, Nutrientes, Nutracêuticos, Vitaminas e Antioxidantes. Foi realizada a busca dos artigos publicados nos últimos 10 anos.
RESULTADOS: Foram selecionados 102 ...
