Navegando por Palavras-chave "taxia-telangiectasia"

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    Efeitos da estimulação do fluxo aéreo nasal na deglutição em pacientes com ataxia-telangiectasia
    (Universidade Federal de São Paulo (UNIFESP), 2014-08-19) Nishinaka, Maria Lucia Tomoko [UNIFESP]; Carvalho, Beatriz Tavares Costa Carvalho [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)
    Ataxia-Telangiectasia - AT is a rare neuromuscular disease caused by a mutation in the chromosome-encoded 11q22-23 ATM genes. Clinical manifestations included: progressive ataxia, oculocutaneous telangiectasia, ocular apraxia, immunodeficiency, recurrent sinopulmonary infections, increased risk for development of malignancy, hypersensitivity to radiation, dysarthria and dysphagia. Despiste the complexity of clinical manifestations, the patients present a progressive muscle impairment, involving the orofacial and respiratory muscles resulting in significant decline in lung function and worsening of dysphagia in these patients. Speech therapy may help to improve the orofacial functions with improved dysphagia (swallowing disorders) in various degenerative diseases such as Parkinson's, Amyotrophic Lateral Sclerosis, Machado-Joseph disease, Friedreich's ataxia and olivopontocerebellar atrophy. To date there aren?t any speech-language studies that show its effects in patients with AT. Furthermore, our professional experience suggests that speech therapy can help in improving the quality of life of individuals, leading us to the challenge of promoting this scientific study. Objective: This study aims to evaluate the effectiveness of speech therapy adapted for patients with AT, with purpose to improve their respiratory function, and its effects on swallowing-related quality of life after 18 months of treatment. Method: The study was conducted on ten patients: five who had been treated weekly (Group S) and five, monthly (Group M). All underwent speech evaluation and the parent or guardian responded jointly with their children to a quality of life questionnaire for dysphagia SWAL-QOL at baseline and after 18 months of speech therapy adapted for these patients. A NFIP (Peak Nasal Inspiratory Flow), and MIP (maximum inspiratory pressure) were performed at the beginning 0, 6, 12 and 18 months. Results: During treatment the improvement of nasal patency and inspiratory muscle strength was observed over time in both groups, but higher in Group S. After treatment the Group S showed a significant improvement in areas such as swallowing burden, symptoms and mental health, compared to the initial results, resulting in improving the quality of life of these patients. The Group M showed improvement but not significant in any of the areas, however, initially have had a better swallowing-related quality of life compared with Group S. All reported improvement in breathing, swallowing and ease of chewing. Conclusion: We conclude that the speech therapy adapted for patients with Ataxia Telangiectasia was effective in the improvement of oral functions, especially respiration and swallowing, assisting in improving the quality of life.