Comprehensive approach to weaning in difficult-to-wean infantile and juvenile-onset glycogen-storage disease type II patients: a case series

Table 3 Changes in respiratory and muscle strength of P 2 (juvenile-onset) after enzyme replacement therapy

Months of ERT (28 days/month)	Proportion of ventilator time			MRC scale
Months of ERT (28 days/month)	Invasive BIPAP%	Invasive CPAP%	NIV%	UL	LL
0.0	100	0	0	4	3
0.5	29	71	0	4	3+
1.0	21	79	0	4	3+
1.5	14	86	0	4+	3+
2.0	0	90	10	4+	3+
2.5	14	50	36	4+	3+
3.0	0	0	100	4+	3+
3.5	0	0	100	5-	3+
4.0	0	0	50^a	5-	3+

ERT enzyme replacement therapy, P patient no, BIPAP Biphasic positive airway pressure, CPAP Continuous positive airway pressure, NIV noninvasive ventilation, MRC medical research council, UL Upper-limb muscle strength, LL lower-limb muscle strength
^aat night

ISSN: 1824-7288