Keywords:
Lung, Respiratory system, Thorax, CT, CT-High Resolution, Diagnostic procedure, Dosimetry, Outcomes analysis, Genetic defects, Cysts, Tissue characterisation
Authors:
K. Weber, M. Paolini, M. Schmitz, M. F. Reiser, U. Mueller-Lisse; Munich/DE
DOI:
10.1594/ecr2013/C-1099
Purpose
Cystic fibrosis (CF),
caused by mutations in a single large gene on chromosome 7 [1] [2],
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 [3] [4].
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 [5].
For analyzing CF-related alterations in lung structure,
computed tomography (CT) has become the diagnostic gold standard.
The benefit of a more exact evaluation is likely to be much higher than the risk of radiation- induced damage [6].
In addition,
the high morphologic accuracy of low-dose multidetector-row CT (LDCT) in benign lung disease has been shown in a recent study [7].
In order to support the assessment of alterations of lung structure in CF patients,
a few high resolution-(HR)-CT based scoring systems,
for example the Brody Score,
with distinct morphologic parameters and different weighting of results,
have been developed [8] [9] [10] [11] [12].
Due to the increasing life expectation of patients suffering from CF,
it seems to be essential to modify scoring systems,
once created for children,
to evaluate the course of CF-related lung damage in adults and to ensure optimal radiological patient management beyond childhood [13].
The aim of this study was to investigate the long-term and short-term intra- and inter-observer reproducibility among three independent readers with different professional experience in diagnostic radiology of the Brody Score in LDCT examinations performed in inspiration of adults suffering from CF related lung disease.