Table 2.

Appropriateness of recommendations according to the GRADE method for outcomes addressed in clinical guidelines for the rehabilitation management of motor and respiratory impairments in patients with late-onset Pompe disease.

Clinical Guidelines (Author, date, reference)	Level of evidence for rehabilitation management	Grade-like recommendations based on level of evidence
Kishnani et al. 2006(3)	3	Submaximal, functional, and aerobic exercise may improve muscle function Gentle daily stretching, orthotic intervention, splinting, seating systems and standing supports may prevent or minimize contracture and deformity
Barba-Romero et al. 2012(29)	3	Aerobic exercise may improve motor function
Cupler et al. 2012(26)	3	Submaximal aerobic exercise, incorporating functional activities may increase muscle strength Daily stretching, orthotic devices, appropriate seating position in the wheelchair, and standing supports may prevent or slow the development of muscle contractures and deformities
Boentert et al. 2016(1)	3	Chest physiotherapy and MAC may be sufficient only for patients with mild expiratory muscle weakness MAC techniques should be implemented by trained physiotherapists or respiratory therapists Air stacking combined with MAC is recommended if cough assistance is indicated and upper airways are patent in cooperative patients I/E devices are indicated if MAC/air stacking are not feasible or ineffective HFCWO is indicated if MAC/air stacking are either not feasible or ineffective and I/E cannot be tolerated
Llerena Junior et al. 2016(30)	2-	Aerobic and progressive resistance exercise training, incorporated into daily functional activities, with or without ERT, may improve muscle strength and functioning Orthotic devices and posture correction while the patient is in the wheelchair and support for when the patient stands may prevent joint contractures
Tarnopolsky et al. 2016(32)	2+	Tailored endurance exercise and progressive resistance training, with or without ERT, may improve aerobic capacity and normalize muscle strength, motor function, and lean mass

Abbreviations: ERT, enzyme replacement therapy; GRADE, Grades of Recommendation, Assessment, Development and Evaluation Working Group; HFCWO, high frequency chest wall oscillation; I/E, Insufflation/Exsufflation; MAC, manually-assisted cough.