Table 2.

Pompe disease related patient characteristics

	Pompe
	N = 17
Age at diagnosis, years	31 ± 14
Disease duration, years	19 ± 14
Hypoventilation, n (%)	9 (53)
Mechanical ventilatory support, n (%)	8 (47)
Severely impaired FVC*, n (%)	7 (44)
Limb girdle weakness, n (%)	16 (94)
Walking aid necessary, n (%)	3 (18)
Six minute walk test, m	410 (234–512)
Enzyme replacement therapy, n (%)	12 (71)
Duration of enzyme therapy, months	72 (29–83)

* - <50% of the predicted value; FVC-upright forced vital capacity