ATROFIA MUSCULAR ESPINHAL PDF

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La atrofia muscular espinal es una enfermedad neurodegenerativa, que puede presentarse con insuficiencia respiratoria progresiva. De los 53 pacientes elegibles, 21 participaron del estudio. Spinal muscular atrophy is a neurodegenerative disorder, which may be associated with progressive respiratory failure.

Our aim is to describe the peak cough flow of children and young people with spinal muscular atrophy types II and III. This is a descriptive, cross-sectional study conducted at a neuropediatrics outpatient clinic between March and May , with patients with spinal muscular atrophy types II and III, and aging more than 5 years.

Out of the 53 eligible patients, 21 participated in the research. The measurement of peak cough flow was carried out through the peak flow meter, with patients sitting and lying down. After taking three measures, we selected the one with the highest value among them. Duas pesquisadoras treinadas e capacitadas aplicaram os testes segundo as diretrizes da European Respiratory Society 22 - Marques et al. Jeong e Yoo 30 corroboraram esse achado.

Arq Neuropsiquiatr. Russman BS. Spinal muscular atrophy: clinical classifications and disease heterogeneity. J Child Neurol. Ann Neurol. Consensus statement for standard of care in spinal muscular atrophy. Spinal muscular atrophy: a clinical and research update. Pediatr Neurol. Kang SW. Pulmonary rehabilitation in patients with neuromuscular disease. Yonsei Med J. Physical dynamics of the cough mechanism. J Appl Physiol. Mechanical aspects of coughing. Sharma GD. Pulmonary function testing in neuromuscular disorders.

Fisioter Mov Curitiba. Clearance of mucus by simulated cough. Iannaccone ST. Modern management of spinal muscular atrophy. Gozal D. Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy. Pediatr Pulmonol. Spinal muscular atrophy: survival pattern and functional status.

Arch Dis Child. Neurologic status, cough, secretions and extubation outcomes. Int Care Med. Respiratory muscle weakness during upper respiratory tract infections. Am Rev Respir Dis. Cough augmentation in Duchenne muscular dystrophy. Am J Phys Med Rehabil. Bach JR. Management of patients with neuromuscular disease. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Peak flow and peak cough flow in the evaluation of expiratory muscle weakness and bulbar impairment in patients with neuromuscular disease.

J Pneumol. Standardisation of spirometry. Eur Respir J. Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure: a different approach to weaning. Bianchi C, Baiardi P. Cough peak flows: standard values for children and adolescents. Assisted cough and pulmonary compliance in patients with Duchenne muscular dystrophy. Monitoring recovery from diaphragm paralysis with ultrasound. Supine fall in lung volumes in the assessment of diaphragmatic weakness in neuromuscular disorders.

Arch Phys Med Rehabil. Air stacking: effects on pulmonary function in patients with spinal muscular atrophy and in patients with congenital muscular dystrophy. J Bras Pneumol. Jeong J, Yoo W. Effects of air stacking on pulmonary function and peak cough flow in patients with cervical spinal cord injury. J Phys Ther Sci. Oskoui M, Kaufmann P. Spinal muscular atrophy. Perspectives on clinical trials in spinal muscular atrophy. Services on Demand Journal. RESUMEN La atrofia muscular espinal es una enfermedad neurodegenerativa, que puede presentarse con insuficiencia respiratoria progresiva.

Recebido: 29 de Maio de ; Aceito: 17 de Outubro de How to cite this article.

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