| | Data |
|---|
Age at PF (years) | 32 (8–65) |
Age at BMF (years) | 20 (1–65) |
Male | 13/18 (72.2%) |
Smoker | 8/17 (47.1%) |
BMF | 20/24(83.3%) |
Mild | 5 (25%) |
Moderate | 3 (15%) |
Severe | 12 (60%) |
DC triad features* | |
0–1 | 3/18(16.7%) |
2–3 | 15/18 (83.3%) |
Family history of DC | 8/16 (50%) |
PFT | |
FEV1, %pred † | 47 ± 15 |
FVC, %pred ‡ | 47 ± 20 |
FEV1/FVC ratio§ | 93 ± 7 |
TLC, %pred§ | 54 ± 14 |
DLCO, %pred|| | 38 ± 14 |
- Data are presented as mean (range) or mean ± standard deviation or number/total number of patients with available data (%). PF: pulmonary fibrosis; BMF: bone marrow failure
- DC: dyskeratosis congenital; PFT: pulmonary function test; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; TLC: total lung capacity; DLCO: diffusing capacity for carbon monoxide
- *DC triad features refer to oral leukoplakia, dysplastic nails and abnormal skin pigmentation
- †FEV1 was available in 9 patients
- ‡FVC was available in 11 patients
- §FEV1/FVC and TLC was available in 8 patients
- ||DLCO was available in 15 patients
