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Table 4 Comparison of DC Patients with Pulmonary Disease with and without HSCT*

From: Lung transplantation for pulmonary fibrosis in dyskeratosis congenita: Case Report and systematic literature review

Parameter

HSCT

No HSCT

**P value

Number with pulmonary disease/total number***

12/70

37/488

0.03

Odds Ratio for pulmonary disease vs. no pulmonary disease, in patients with HSCT

2.2 (1-4.5)

-

0.03

Age (years) at pulmonary disease in those who developed pulmonary disease, median (range)

14 (4-36)

37 (7-68)

<0.001

Median survival age (years) free of pulmonary disease

34

61

<0.001

Interval (years) from HSCT to pulmonary disease, median (range)

4.5 (0-20)

-

-

Number who died

9/12

24/37

0.8

Interval (years) from pulmonary disease to death in those who developed pulmonary disease, median (range)

0.3 (0-2)

0 (0-8)

0.5

Median survival interval (years) after pulmonary disease,

1

2

0.3

  1. * Data obtained from the cases in Tables 2 and 3.
  2. **P values compare the patients who received HSCT with those who did not receive HSCT.
  3. *** The denominator is the total number of patients with DC who did or did not receive HSCT.
  4. Abbreviations: DC, dyskeratosis congenita; HSCT, hematopoietic stem cell transplantation.