title: Pulmonary emphysema in cystic fibrosis detected by densitometry on chest multidetector computed tomography.
abstract: BACKGROUND
  Histopathological studies on lung specimens from patients with cystic fibrosis (CF) and recent results from a mouse model indicate that emphysema may contribute to CF lung disease. However, little is known about the relevance of emphysema in patients with CF. In the present study, we used computationally generated density masks based on multidetector computed tomography (MDCT) of the chest for non-invasive characterization and quantification of emphysema in CF.
  
  
  METHODS
  Volumetric MDCT scans were acquired in parallel to pulmonary function testing in 41 patients with CF (median age 20.1 years; range 7-66 years) and 21 non-CF controls (median age 30.4 years; range 4-68 years), and subjected to dedicated software. The lung was segmented, low attenuation volumes below a threshold of -950 Hounsfield units were assigned to emphysema volume (EV), and the emphysema index was computed (EI). Results were correlated with forced expiratory volume in 1 s percent predicted (FEV1%), residual volume (RV), and RV/total lung capacity (RV/TLC).
  
  
  RESULTS
  We show that EV was increased in CF (457±530 ml) compared to non-CF controls (78±90 ml) (P<0.01). EI was also increased in CF (7.7±7.5%) compared to the control group (1.2±1.4%) (P<0.05). EI correlated inversely with FEV1% (rs=-0.66), and directly with RV (rs=0.69) and RV/TLC (rs=0.47) in patients with CF (P<0.007), but not in non-CF controls. Emphysema in CF was detected from early adolescence (~13 years) and increased with age (rs=0.67, P<0.001).
  
  
  CONCLUSIONS
  Our results indicate that early onset emphysema detected by densitometry on chest MDCT is a characteristic pathology that contributes to airflow limitation and may serve as a novel endpoint for monitoring lung disease in CF.
authors.affiliation: Department of Diagnostic and Interventional Radiology, University of Heidelberg, Heidelberg, Germany.
authors.forename: Mark O
authors.initials: MO
authors.lastname: Wielpütz
authors.forename: Oliver
authors.initials: O
authors.lastname: Weinheimer
authors.forename: Monika
authors.initials: M
authors.lastname: Eichinger
authors.forename: Matthias
authors.initials: M
authors.lastname: Wiebel
authors.forename: Jürgen
authors.initials: J
authors.lastname: Biederer
authors.forename: Hans-Ulrich
authors.initials: HU
authors.lastname: Kauczor
authors.forename: Claus P
authors.initials: CP
authors.lastname: Heußel
authors.forename: Marcus A
authors.initials: MA
authors.lastname: Mall
authors.forename: Michael
authors.initials: M
authors.lastname: Puderbach
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications*; D004646:Emphysema / Q000150:complications / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed / Q000379:methods*