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Table 2 Pulmonary function tests

From: Severe tracheal and bronchial collapse in adults with type II mucopolysaccharidosis

  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
  1. 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