Abstract
PURPOSE: Morquio's Syndrome (MS) is an inherited metabolic disorder caused by a deficiency of a specific lysosomal enzyme. Patients develop respiratory failure secondary to reduced chest wall compliance, reversible and irreversible airway obstruction, chronic obstructive lung disease and obstructive sleep apnea. Formal respiratory function tests are difficult to perform and to interpret owing to the small size of these patients and to poor patient cooperation. The advent of infant pulmonary function testing (IOS) may identify MS children with significant pulmonary limitations. The aim of this study is to evaluate this hypothesis.
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