| |
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
