Severe tracheal and bronchial collapse in adults with type II mucopolysaccharidosis

Table 2 Pulmonary function tests

	Patient 1	Patient 2	Patient 3	Patient 4	Patient 5
VC max (L)	4.01	3.79	1.83	1.5	2.01
VC max (%)	91	78	46	40	61
FEV1 (L)	1.32	2.33	1.36	0.55	1.18
FEV1 (%)	38	62	43	18	41
Tiffeneau (FEV1/VC) (%)	33	61	74	37	59
TLC-He (L)	5.52	5.38	2.84	3.6	2.94
TLC-He (%)	84	77	49	71	66
TLC-Pleth			3.50
TLC-Pleth (%)			60
DLCO (%)	73	94	46	44	43
DLCO/VA(%)	108	140	132	109	129
Age at time of start ventilatory support	50	N/A	40	31	N/A
Type of ventilatory support	CPAP	N/A	BiPAP	CPAP	N/A

VC vital capacity (in litre and as % of predicted), FEV1 forced expiratory volume in 1 s (in litre and as % of predicted), TLC-He total lung capacity measured by helium dilution (in litre and as % of predicted), TLC-Pleth total lung capacity measured by bodyplethysmography (in liter and as % of predicted), DLCO carbon monoxide diffusing capacity (% of predicted), DLCO/VA carbon monoxide diffusing capacity adjusted for lung volume (% of predicted). CPAP continuous positive airway pressure, BiPAP bilevel positive airway pressure

ISSN: 1750-1172