Cough Effectiveness and Pulmonary Hygiene Practices in Patients with Pompe Disease

摘要

Purpose While factors leading to hypoventilation have been well studied in Pompe disease, cough effectiveness and airway clearance practices are less understood. We aimed to identify significant factors that influence peak cough flow (PCF) in Pompe, and to detect whether pulmonary hygiene practices were reflective of reduced PCF.