História Natural da Deglutição e Linguagem na Mucopolissacaridose II. / Natural History of Language and Swallowing in Mucopolyssacaridoses.

AUTOR(ES)
DATA DE PUBLICAÇÃO

2010

RESUMO

Purpose: To analyze the evolution of language and swallowing alterations of patients diagnosed with mucopolysaccharidosis type II after one year of the first assessment, and to observe the influence of the variables age, severity of the disease and impairment of breathing. Methods: Seven patients with mucopolysaccharidosis II, two with light type and five with severe type, all of them recorded at the Genetics Departament of the UNCISAL. Patients under enzyme replacement therapy and/or alternative feeding were excluded. Data collection was divided into two stages: the first one from December 2007 to January 2008, and the second one was reapplied after one year. The aspects of receptive-hearing language of the Peabody test, and the pragmantic aspect following ABFW test were evaluated. After that, it was applied the face anthropometric protocol, and realized speech pathology clinical evaluation. The swallowing evaluation focused on dysphagia using the clinical and fiberoptic endoscopic examination. After these evaluations,the patients were classified according to the Commitment Functional Scale of Swallowing after fiberoptic endoscopic examination and the Functional Oral Intake Scale FOIS. Results: All patients showed impairment in face anthropometric and phonoarticulatory organs and the functions of the stomatognathic system. There was no statistically significant difference between the evaluations considering the period of time, except for the lower lip. Swallowing evaluation showed that two of the severe type and three of light type presented dysphagia with impact on oral feeding, with negative variation in the results after one year on the Fois scale for the light type. All showed impairment regarding language with more severe impairment in the severe type. Regarding correlation between variables it was observed worsening of the respiratory changes, of the level of Fois scale and improvement of the respiratory impact positively on VED, and weak positive correlation between pragmatic and receptive vocabulary. The second assessment showed only a weak negative correlation between breathing and the level of the Fois scale. Conclusions: 1. There was significant change in pragmatic language and receptive vocabulary among severe and light type in both evaluations. 2. Swallowing had negative impact on oral feeding, with indication of non oral food ingestion in severe type; the light type showed minor variations regarding oral feeding. 3. There was no evidence of positive or negative correlation between language and swallowing after one year, but there was a correlation between worsening of breathing and swallowing.

ASSUNTO(S)

fonoaudiologia 1. mucopolysaccharidosis ii 3. language, 4. swallowing, 5. deglutition disorders. 1. mucopolissacaridose ii. 2. linguagem, 3. deglutição. 4. transtorno de deglutição.

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