Cystic fibrosis (CF),
caused by mutations in a single large gene on chromosome 7  ,
is the most abundant life-shortening autosomal recessive disease and shows a carrier frequency of about 4-5% as well as an incidence about one in 2,500 live births  .
Lifespan among patients suffering from CF has increased from about 25 years in 1985 to 37 years in 2008,
because of new groundbreaking therapeutic options and diagnostic tools . For analyzing CF-related alterations in lung...
Methods and Materials
Repetitive assessment of low-dose MDCT in patients with cystic fibrosis Brody scores and respective bronchiectasis-,
and hyperinflation subscores were assessed twice (time interval,
28 days to 7 years) by each of 3 independent radiologists (R1,
with 1-20 years of professional experience in diagnostic radiology) in low-dose multidetector-row CTs of the chest (LDCT,
Respective median time intervals and ranges between first and second LDCT readings were 4.7 years (2.0-7.0) for R1,
42 days (32-60) for R2,
and 37 days (28-47) for R3. Brody Score (total score,
Results Table 1 ,
Def. Table 7 ) Comparison of individual Brody scoring results implied an average intra-individual reproducibility within about 7% of the individual mean score (Table 1),
with wider deviations for long-term re-assessment by the attending radiologist . There was no evidence of skewing...
The Brody score shows high average inter-observer reproducibility in adult patients undergoing inspiratory-phase LDCT for CF-related lung disease. The Brody score also demonstrates high average intra-observer reproducibility in adult patients undergoing inspiratory-phase LDCT for CF-related lung disease,
if the time period between the two readings of the same examinations ranges between 28 and 61 days.
With an increasing time interval between the two evaluations,
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