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Table. 1 Demographics and clinical characteristics of patients with DC-related PF (n = 27)

From: Pulmonary fibrosis in dyskeratosis congenita: a case report with a PRISMA-compliant systematic review

  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
  1. 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
  2. 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
  3. *DC triad features refer to oral leukoplakia, dysplastic nails and abnormal skin pigmentation
  4. FEV1 was available in 9 patients
  5. FVC was available in 11 patients
  6. §FEV1/FVC and TLC was available in 8 patients
  7. ||DLCO was available in 15 patients