Table 1—

Demographic data and diagnoses of all randomised patients and those that completed the protocol

EnrolledCompleted protocol
Male/female n18/714/6
Age yrs42±17.742±17.6
Pa,O2 kPa11.6±1.911.7±2.0
Pa,CO2 kPa5.9±0.95.8±1.0
FVC L0.89±0.40.89±0.4
FVC % pred22.4±11.022.4±9.3
FEV1 L0.69±0.40.68±0.3
FEV1 % pred20.8±11.720.3±9.2
FEV1/FVC0.77±0.140.77±0.13
PI,max cmH2O34±17.433±15.9
PE,max cmH2O58±39.362±40.1
Pmo,w cmH2O58±45.861±48.7
SNIP cmH2O26±12.726±10.9
Diagnosis n
 Duchenne muscular dystrophy43
 Congenital muscular dystrophy (merosin positive)21
 Old poliomyelitis33
 Spinal muscular atrophy type II21
 Idiopathic scoliosis98
 Osteogenesis imperfecta11
 Congenital myopathy11
 Nemaline myopathy11
 Charcot–Marie tooth neuropathy10
 Idiopathic diaphragm paralysis11
  • Data are expressed as mean±sd, unless otherwise stated. Pa,O2: arterial oxygen tension; Pa,CO2: arterial carbon dioxide tension; FVC: forced vital capacity; % pred: % predicted; FEV1: forced expiratory volume in one second; PI,max: maximal inspiratory pressure at mouth; PE,max: maximal expiratory pressure at mouth; Pmo,w: maximal whistle mouth pressure; SNIP: sniff nasal inspiratory pressure. 1 cmH2O = 0.098 kPa.