Acute Airway Crisis in Mucopolysaccharidosis VI: Management Challenges

Abstract excerpt

<h4>Objectives: </h4> Mucopolysaccharidosis type VI is rare lysosomal storage disorder due to arylsulfatase B enzyme deficiency, leading to progressive multisystem disease and complex airway. Acute respiratory infections can precipitate airway embarrassment. A structured treatment guideline is currently lacking. We present 7 years old MPS VI male with respiratory distress highlighting the challenges in management....

Topics

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• Carbohydrate Chemistry and Synthesis
• Disease Models, Animal
• Early Diagnosis
• Iduronidase
• Lysosomal Storage Disorders Research
• Mucopolysaccharidosis I
• Phenotype
• Proteoglycans and glycosaminoglycans research

Identifiers and source

Literature Corpus work

6fe43141-e817-5b51-8709-5a8e72789866

DOI

10.20944/preprints202602.1905.v1