Table 1.

Protocol for glycogenosis type II.

	Pre	to	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W	W
	ERT		2	4	6	8	10	12	14	16	28	20	22	24	26	28	30	32	34	36	38	40	42	44	46	48	50	52
Informed consent	X
Inclusion criteria	X
Medical history	X
Nutritional evaluation	X														X													X
6MWT	X	X			X			X							X						X							X
FVC	X	(X)						X							X						X							X
MMT	X	X						X							X						X							X
Functional activity	X							X							X						X							X
(GSGC)
RHS
SF-36 Health survey	X														X						X							X
Gene GAA mutation	X
GAA activity	X
in muscle
Vital signs	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X
Blood exams	X	X	X	X		X		X							X						X							X
Urine exams	X	X	X	X		X		X		X		X		X		X		X		X		X		X		X		X
EGG	X														X													X
Echocardiography	X																											X
Weight	X							X							X						X							X
Anti-rhGAA		X		X		X		X		X		X		X		X		X		X		X		X		X		X
Antibody-IgG
Drug infusion		X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X	X
Ventilation diary	Continuous
	monitoring
Adverse reactions	Continuous
	monitoring

6MWT, six-minute walk test; FVC, forced vital capacity; MMT, manual muscle testing; RHS, Rotterdam Handicap Scale; ECG, electrocardiogram.