Prospective studies of swallowing in Mucopolysaccharidosis II (Hunter syndrome) before and after enzyme treatment

Revista da Sociedade Brasileira de Fonoaudiologia

Ferreira, Ana Carolina Rocha Gomes¹  Universidade Federal de São Paulo, São Paulo, Brasil¹  Guedes, Zelita Caldeira Ferreira¹  Universidade Estadual de Ciências da Saúde de Alagoas, Maceió, Brasil¹  Universidade Federal de São Paulo¹ 

关键词: Deglutition disorders;    Mucopolysaccharidosis II;    Therapeutic;    Case studies;    Prospective studies;   

DOI  :  10.1590/S1516-80342011000200018

来源: Sociedade Brasileira de Fonoaudiologia

【 摘 要 】

Case study with the aim to evaluate swallowing pre- and post-enzyme treatment of an individual with Mucopolysaccharidosis, and to analyze whether this treatment caused swallowing improvement. It was carried out a descriptive study of a male subject with 9 years and 6 months with the severe type of the disease. The subject was observed in three distinct moments: one pre-treatment and two post-treatment assessments. It was carried out a speech-language pathology assessment of dysphagia, a pneumologist's respiratory assessment, a clinical otorhinolaryngology assessment, and a fiberoptic endoscopic examination of swallowing. After that, results were interpreted according to the Functional Oral Intake Scale (FOIS), and data were descriptively analyzed. Results showed presence of dysphagia with impact on oral feeding in the pre-treatment and the first post-treatment assessments. The second post-treatment assessment did not show any signs of dysphagia, with possibilities for all consistencies, however with restriction to solid consistency due to a significant occlusal alteration. Moreover, the fiberoptic endoscopic examination of swallowing was normal in this assessment, and there was a gradual improvement on the swallowing dynamics along the treatment. The study showed that dysphagia was present and had a negative impact on food dynamics for the subject studied, and also that the enzyme treatment for over a year improved swallowing, with positive impact on food dynamics.

【 授权许可】

Unknown