The impact of laronidase treatment in otolaryngological manifestations of @entity1 with XXXX .

@entity2315 @entity1 @entity2167 @entity1184 @entity2972 @entity281 @entity5254 @entity1646 @entity12751 @entity1576 @entity1729 @entity8408 @entity393 @entity11124 @entity2346
INTRODUCTION: @entity1646 ( @entity1646 ) is a @entity2315 caused by deficiency of a-l-iduronidase. The otolaryngological findings include @entity393 , @entity2972 , recurrent @entity2346 , @entity1184 and adenoid, recurrent rhinosinusitis, @entity8408 , snoring, oral breathing and nasal obstruction. OBJECTIVE: To evaluate the impact of enzymatic replacement therapy with laronidase (Aldurazyme(  )) in @entity1 with @entity1646 ( @entity12751 ), regarding sleep and @entity2167 , and clinical manifestations in the upper respiratory tract (URT). METHODS: Nine @entity1 with @entity12751 (8 @entity12751 , and 1 Scheie phenotypes) of both sexes, ages ranging between 3 and 20 years, were included in this study. @entity1 were evaluated between seven and 11 months before the treatment and between 16 and 22 months after the onset of the enzymatic replacement. They were all submitted to a clinical and otolaryngological evaluation, including nasofibroscopical, polysomnographic and audiologic exams. RESULTS: The results' data showed decreasing of the frequency of ear, nose and @entity281 , with improvement of the @entity5254 and @entity1576 . No remarkable changes were observed regarding @entity11124 and tonsil and @entity1184 . Audiometric and polysomnographic evaluations did not show statistical significance. CONCLUSION: Enzymatic replacement therapy in @entity1 with @entity12751 provides control of recurrent @entity1729 , @entity5254 and @entity1576 , however it is does not seem to improve audiologic and polisomnographic parameters, with no effect on adenoid and tonsils @entity1184 and @entity11124 .
