Pulmonary function
| Patient | 1 | 2 | 3 | 4 | 5 | 6 | Mean age |
| Age (y) 1st RFT | 5 | 5 | 5 | 4 | 5 | 4 | 4 years and 7 months |
| Age (y) last RFT | 8 | 7 | 8 | 7 | 7 | 6 | 7 years |
| Mean RFT | |||||||
| 1st FVC | 74% | 55% | 50% | 88% | 47% | 75% | 65% |
| Last FVC | 66% | 59% | 57% | 80% | 59% | 99% | 70% |
| 1st FEV1 | 79% | 37% | 37% | 66% | 49% | 85% | 59% |
| Last FEV1 | 63% | 34% | 30% | 76% | 46% | 82% | 55% |
| 1st peak flow | 97% | 43% | 55% | 58% | 65% | 101% | 70% |
| Last peak flow | 66% | 34% | 36% | 62% | 51% | 85% | 56% |
Values in per cent predicted for height.8
RFT, respiratory function test.