Progression of PFTs
The total number of PFTs was different for each patient (median 6;
range:2-17). At each PFTs, FVC, FEV1,
FEV1/FVC and MMEF25%-75% were all
performed in all patients. The median time interval between each PFTs
was 9 months (range: 1-35 months). There was significant inter- and
intra-individual variability in every PFT parameter over time (Figure
2). However, even after taking that into account, over time, the FVC and
FEV1 improved in eleven children and was only mild
decrease in one child, FVC and FEV1 increased by a mean
of 8.212%/year (95% CI: 6.531%-9.894%; p<0.0001) and
5.007%/year (95% CI: 3.463%-6.552%; p<0.0001) (Table 2,
Figure 2). But the increase in FEV1 was not as
significant as FVC, so there was a unanimous and significant fall in
FEV1/FVC ratio with an average decrease of 3.537%/year
(95% CI: 1.984%-5.09%; p<0.0001) (Table 2, Figure 2).
MMEF25%-75% improved in nine children and unchanged or
declined slightly in three children, resulting in an average increase of
1.583% per year (95% CI: 0.046%-3.12%; p<0.05) (Table 2,
Figure 2).
Over all, FEV1 and MMEF25%-75% showed
different degrees of improvement after inhaled bronchodilators at each
PFT sessions for ten patients, and FEV1 was with
significant (>12%) β2-bronchodilation in
53% of PFT sessions. (Figure 2, data was not given).