Respiratory aspects and pulmonary physiotherapy in the child with mucopolysaccharidosis

Abstract

Mucopolysaccharidosis is a heterogeneous group of diseases characterized by the lysosomal accumulation of intermediates in the metabolism of mucopolysaccharides or glycosaminoglycans. The respiratory disorder that characterizes patients with mucopolysaccharidosis is the chronic restrictive lung disease. Therefore, pulmonary rehabilitation aimed at improving respiratory symptoms and respiratory physiotherapy will improve pulmonary ventilation and impaired respiratory biomechanics in patients with mucopolysaccharidosis. Follow-up by the pediatric pulmonologist is necessary to quantify lung function and monitor the symptoms of nocturnal obstruction and pulmonary restriction with the help of studies such as spirometry, ple-thysmography and the 6-minute walk test, among others. It is also very important to perform an individualized program of respiratory physiotherapy techniques and exercises. Overall, all of these steps are followed for evaluating lung function as a marker of response to the use of any of the therapies indicated in mucopolysaccharidosis.