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Table. 3 Clinical characteristics of patients with early-onset or later-onset PF (n = 27) and with different gene mutations (n = 16)

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

Variable

Early-onset (n = 16)

Later-onset (n = 11)

P

TERC/TERT (n = 5)

TINF2 (n = 6)

DKC1/NHP2 (n = 5)

P

Age at BMF* (year)

9 ± 8

30 ± 23

0.017

43 ± 14

4 ± 4

23 ± 21

0.003†

Age at PF (year)

/

/

 

56 ± 8

24 ± 13

36 ± 16

0.004‡

BMF (moderate to severe)

13/16 (81.3)

2/8 (25.0)

0.021

1/2 (50.0)

4/6 (66.7)

3/5 (60.0)

1.000

CT patterns

       

UIP/possible UIP

7/15 (46.6)

6/11 (54.5)

1.000

4/5 (80.0)

3/6 (50.0)

2/5 (40.0)

 > 0.05#

  1. Data are presented as mean ± standard deviation or number/total number of the patients with available data (%)
  2. DC: dyskeratosis congenital; BMF: bone marrow failure; PF: pulmonary fibrosis; CT: computed tomography; UIP: unusual interstitial pneumonia;/: not applicable
  3. *Age at BMF was unavailable in 1 patient with later-onset PF and 1 patient with TINF2 variants
  4. †TERC/TERT versus TINF2, P = 0.006; TINF2 versus DKC1/NHP2, P = 0.277; TERC/TERT versus DKC1/NHP2, P = 0.266
  5. ‡TERC/TERT versus TINF2, P = 0.001; TINF2 versus DKC1/NHP2, P = 0.137; TERC/TERT versus DKC1/NHP2, P = 0.028
  6. #TERC/TERT versus TINF2, P = 0.545; TINF2 versus DKC1/NHP2, P = 1.000; TERC/TERT versus DKC1/NHP2, P = 0.524
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BMC Pulmonary Medicine

ISSN: 1471-2466

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