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View article: Novel approach for tracheal resection in Morquio a syndrome with end-stage critical airway obstruction: a UK case series
Novel approach for tracheal resection in Morquio a syndrome with end-stage critical airway obstruction: a UK case series Open
Background Mucopolysaccharidosis (MPS) type IVA is a rare lysosomal storage disorder caused by aberrations of the N-acetyl-galactosamine-6-sulfatase (GALNS) enzyme. MPS IVA is associated with a wide gamut of respiratory and airway disorder…
View article: Novel Approach for Tracheal Resection in Morquio A Syndrome with End-stage Critical Airway Obstruction: A UK Case Series
Novel Approach for Tracheal Resection in Morquio A Syndrome with End-stage Critical Airway Obstruction: A UK Case Series Open
Background Mucopolysaccharidosis (MPS) type IVA is a rare lysosomal storage disorder caused by aberrations of the N-acetyl-galactosamine-6-sulfatase (GALNS) enzyme. MPS IVA is associated with a wide gamut of respiratory and airway disorder…
View article: Tracheal Resection for Critical Airway Obstruction in Morquio A Syndrome
Tracheal Resection for Critical Airway Obstruction in Morquio A Syndrome Open
Introduction . The primary cause of death in Morquio A syndrome (mucopolysaccharidosis (MPS) IVA) is airway obstruction, brought about by an inexorable and pathognomonic multilevel airway tortuosity, buckling, and obstruction. The relative…
View article: The characterisation of pulmonary function in patients with mucopolysaccharidoses IVA: A longitudinal analysis
The characterisation of pulmonary function in patients with mucopolysaccharidoses IVA: A longitudinal analysis Open
Whilst spirometry values showed a gradual decline across all groups, oximetry showed modest improvement in respiratory function. The amalgamation of FEV1, FVC [%predicted] and ODI 3% appeared predictive of changes in respiratory function i…