Table 1.
Pulmonary function at diagnosis of pulmonary fibrosis, before and after lung transplantation
| Age in years | 11 6/12 | 11 9/12 | 12 9/12 | 14 6/12 |
|---|---|---|---|---|
| Pulmonary function tests | At presentation | After steroid burst | 3 months prior to lung transplantation | 18 months after lung transplantation |
| FVC (L), (% predicted) | 0.78; (31%) | (51%) | 0.51; (20%) | 2.29; (81%) |
| FEV1 (L), (% predicted) | 0.73; (33%) | (48%) | 0.51, (24%) | 1.91; (78%) |
| FEV1/FVC% | 0.94; (109%) | - | 100% | 83% |
| DLCO (corrected) | - | 27% | - | 85% |
| TLC (L) | - | 56%) | 1.45; (41%) | 2.36; (81%) |
| FEF 25-75% (L/second) | 0.88; (35%) | 1.29; (52%) | 2.08; (81%) | |
| RV/TLC (% predicted) | - | 33% | 53% | - |
| PEFR; (% predicted) | 2.86; (61%) | - | 1.74; (36%) | - |
| VQ scan | - | - | Moderate or large areas of perfusion defect | Normal |
| Lung CT | Interstitial fibrosis | - | Diffuse fibrosis and ground glass opacities. Subpleural reticulation. Bilateral traction bronchiectasis. | Mild atelectasis |
| Lung biopsy | Pulmonary fibrosis | - | - | No rejection |
Abbreviations: CT, computerized tomography; DLCO, diffusion capacity for carbon monoxide; FEF, forced expiratory flow; FEV1, forced expiratory volume in 1 second; FVC, forced vital capacity; HSCT, hematopoietic stem cell transplant; PEFR, peak expiratory flow rate; RV, residual volume; TLC, total lung capacity; VQ scan, pulmonary ventilation/perfusion scan.