Table 2.
FIRST test | LAST test | |||||
---|---|---|---|---|---|---|
na (%) | Median (IQR) | na (%) | Median (IQR) | Mean difference | p valueb | |
Follow-upc [years] | 3.04 (1.23–5.38) | 9.33 (6.08–12.27) | ||||
FEV1 z-score | ||||||
Whole cohort | 72 | −0.76 (−2.12–0.08) | 72 | –1.01 (−2.19–−0.55) | −0.32 (−1.09–0.35) | 0.115 |
Normal | 48 (67) | 47 (65) | ||||
Reduced | 24 (33) | 25 (35) | ||||
FVC z-score | ||||||
Whole cohort | 66 | −0.87 (−2.18–−0.22) | 66 | −1.16 (−2.21–−0.61) | −0.31 (−0.96–0.35) | 0.182 |
Normal | 45 (68) | 41 (62) | ||||
Reduced | 21 (32) | 25 (38) | ||||
FEV1/FVC | ||||||
Whole cohort (ratio) | 66 | 0.93 (0.89–0.97) | 66 | 0.91 (0.87–0.94) | −0.02 (−0.05–0.01) | 0.031 |
Ratio ≥0.7 | 65 (99) | 65 (99) | ||||
Ratio <0.7 | 1 (1) | 1 (1) | ||||
MMEF | ||||||
Whole cohort | 42 | 0.11 (−1.11–0.94) | 42 | −0.07 (−0.98–0.35) | −0.14 (−0.85–0.85) | 0.421 |
Normal | 35 (83) | 39 (93) | ||||
Reduced | 7 (17) | 3 (7) | ||||
TLC z-score | ||||||
Whole cohort | 58 | −1.26 (−2.55–−0.22) | 58 | −0.81 (−2.14–0.23) | 0.15 (−0.97–1.44) | 0.309 |
Normal | 33 (58) | 39 (68) | ||||
Reduced | 25 (42) | 19 (32) | ||||
RV z-score | ||||||
Whole cohort | 55 | 0.55 (−1.22 – 2.05) | 55 | 0.31 (−0.51–1.32) | −0.43 (−1.44–0.87) | 0.521 |
Normal | 49 (89) | 50 (91) | ||||
Reduced | 6 (11) | 5 (9) | ||||
At least one abnormal test | 44 (59) | 38 (51) |
Normal: measured value ≥ −1.645 z-score.
Reduced: measured value < −1.645 z-score.
aTotal number of CCSs differ between lung function parameters as not all survivors had all parameters performed at first and last test.
bWilcoxon rank sum test comparing median z-score between first and last test.
cTime between diagnosis and pulmonary function test.