The impact of laronidase treatment in otolaryngological manifestations of patients with mucopolysaccharidosis.

Introduction: Mucopolysaccharidosis (MPS) is a lysosomal storage disease caused by deficiency of α-l-iduronidase. The otolaryngological findings include hearing loss, otorrhea, recurrent otitis, hypertrophy of tonsils and adenoid, recurrent rhinosinusitis, speech disorders, snoring, oral breathing and nasal obstruction.

Objective: To evaluate the impact of enzymatic replacement therapy with laronidase (Aldurazyme(®)) in patients with mucopolysaccharidosis (MPS I), regarding sleep and hearing disorders, and clinical manifestations in the upper respiratory tract (URT).

Nutritional evaluation in surgical treatment of children with hypertrophic tonsils and or adenoids.

Eighty seven children, between 2 and 10 years of age were studied. Twenty four had adenotonsillar hypertrophy and underwent surgery (Group I). Fifteen had adenoidal hypertrophy and underwent surgery (Group II).