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目的庞贝是一种累及心脏和骨骼肌的糖原累积病,以肌病表现为主,可影响所有主要的呼吸肌,呼吸并发症是主要的致死原因。为了解我国晚发庞贝病患者呼吸功能的状态,为患者呼吸支持提供依据,我们进行了此项研究。方法本研究共纳入11名庞贝病患者,首先进行病史及临床症状问卷。随后进行立卧位流速-容量环、最大呼气压及最大呼气压及咳嗽峰流速的测定。
Purpose Pompeii is a glycogen-accumulating disease involving the heart and skeletal muscles that is predominantly myopathic and affects all major respiratory muscles. Respiratory complications are the leading cause of death. In order to understand the status of respiratory function in patients with late-onset Pompeii in our country and provide the basis for respiratory support, we conducted this study. Methods A total of 11 patients with Pompeii were enrolled in this study. The first was a history and clinical symptom questionnaire. Then set the vertical position of the flow rate - volume ring, the maximum expiratory pressure and maximum expiratory pressure and cough peak flow rate determination.