WS07.4 MBW and MRI as sensitive markers of stable CF lung disease and at exacerbation in children and adolescents
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文摘
MBW and MRI have both been shown to be sensitive outcome measures for CF lung disease. Therefore, we wanted to determine the correlation of the LCI with alterations in the MRI chest score as markers of CF lung disease under stable conditions and at exacerbation.

Methods

57 stable children with CF (mean age, 9.5±6.2 years) had chest MRI scans performed. All children with CF and 106 healthy children (mean age, 8.9±4.8 years) underwent age-adapted MBW testing to assess LCI and to calculate LCI z-scores. 36 children (mean age, 13.9±4.4 years) were investigated at time of pulmonary exacerbation either via MRI (n = 10) or MBW (n = 26).

Results

Mean LCI z-score in healthy children was 0.1±1.2). LCI z-score was significantly higher in stable children with CF (5.4±5.5, P<0.001). Morphological, perfusion and global MRI chest score were elevated in stable children with CF and were positively correlated with a higher LCI z-score (R 0.682; 0.669; 0.711). Concordance between abnormal findings in MBW and MRI was above 70%. At exacerbation, LCI increased in children with CF from baseline (P<0.001) and decreased after antibiotic therapy (P<0.001). The MRI chest scores also decreased after iv antibiotic treatment (P<0.05).

Conclusion

LCI z-score and MRI chest score are elevated in CF and show a high correlation. Both methods are feasible and sensitive non-invasive markers of CF lung disease in children with CF under stable conditions and at time of exacerbation.

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