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.

Giri et al. BMC Blood Disorders 2011 11:3   doi:10.1186/1471-2326-11-3