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 structure,
computed tomography (CT) has...
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,
effective dose appr.
0.5 mSv) performed in inspiration in 15 consecutive adult patients suffering from CF-related lung...
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,
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 of the average deviation toward lower or higher...
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,
the intra-observer reproducibility appears to decline.
The respective subscores of the Brody score each present with a lower intra- and inter-observer...
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Pulmonary abnormalities on...