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  • cystic fibrosis  (8)
  • 1
    Keywords: THIN-SECTION CT ; INFANTS ; CHILDREN ; asthma ; LUNG-DISEASE ; cystic fibrosis ; SCORING SYSTEMS ; DIMENSIONS ; airway dimensions ; airway disease ; CHEST RADIOGRAPH ; chronic obstructive pulmonary disease ; FLOW LIMITATION ; quantitative computed tomography ; WALL THICKNESS
    Abstract: Purpose: To evaluate the fully automatic quantification of airway dimensions on chest multidetector computed tomography (MDCT) performed in cystic fibrosis (CF) patients. Airflow indices including predicted forced expiratory volume in 1 second (FEV1%) were used to study the impact on regional lung function. Materials and Methods: MDCT data of patients with CF (14 children and 23 adults) and of control patients (11 children and 22 adults) were used to compute total diameter (TD), lumen area (LA), and wall thickness (WT) using dedicated software. Pulmonary function testing including FEV1% was performed in parallel and correlated with MDCT parameters in a generation-based analysis. Results: TD was largely increased in CF patients (third-generation to fourth-generation airways in children, first to ninth in adults; P 〈 0.05). LA remained unchanged, but WT was also larger in CF compared with controls (third generation to sixth generation in children, first to eleventh in adults; P 〈 0.05). In adult CF patients significant negative correlations for TD, LA, and WT with FEV1% were found for intermediate airways (fifth to seventh generation; r = -0.7 to -0.9) but not in pediatric CF patients and controls. Conclusions: Automatic airway analysis succeeded in quantifying specific pathologies such as airway dilatation and wall thickening in CF patients at different ages. Moreover, our results indicate a shift in main airflow resistance to intermediate airways in cases of chronic CF. The objective computational parameters TD, LA, and WT should be considered for assessment and follow-up of CF airway disease
    Type of Publication: Journal article published
    PubMed ID: 23222199
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  • 2
    Keywords: Germany ; LUNG ; PERFUSION ; THERAPY ; CT ; FOLLOW-UP ; imaging ; VENTILATION ; DISEASE ; EXPOSURE ; LONG-TERM ; POPULATION ; radiation ; prognosis ; FLOW ; MRI ; PROGRESSION ; MUTATION ; GAS ; MUTATIONS ; FREQUENT ; CHILDREN ; HYPERPOLARIZED HE-3 ; MR imaging ; review ; monitoring ; CHEST-X-RAY ; HUMAN LUNG ; cystic fibrosis ; CHILD ; proton MRI ; INTERVENTIONS ; MODALITY ; Follow up
    Abstract: Cystic fibrosis (CF) lung disease is caused by mutations in the CFTR-gene and remains one of the most frequent lethal inherited diseases in the Caucasian population. Given the progress in CF therapy and the consecutive improvement in prognosis, monitoring of disease progression and effectiveness of therapeutic interventions with repeated imaging of the CF lung plays an increasingly important role. So far, the chest radiograph has been the most widely used imaging modality to monitor morphological changes in the CF lung. CT is the gold standard for assessment of morphological changes of airways and lung parenchyma. Considering the necessity of life-long repeated imaging studies, the cumulative radiation doses reached with CT is problematic for CF patients. A sensitive, non-invasive and quantitative technique without radiation exposure is warranted for monitoring of disease activity. In previous studies, MRI proved to be comparable to CT regarding the detection of morphological changes in the CF lung without using ionising radiation. Furthermore, MRI was shown to be superior to CT regarding assessment of functional changes of the lung. This review presents the typical morphological and functional MR imaging findings with respect to MR-based follow-up of CF lung disease. MRI offers a variety of techniques for morphological and functional imaging of the CF lung. Using this radiation free technique short- and long-term follow-up studies are possible enabling an individualised guidance of the therapy
    Type of Publication: Journal article published
    PubMed ID: 20432002
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  • 3
    Keywords: DIAGNOSIS ; SYSTEMS ; TIME ; REPRODUCIBILITY ; COMPUTED-TOMOGRAPHY ; THIN-SECTION CT ; magnetic resonance imaging (MRI) ; CHILDREN ; ADULTS ; cystic fibrosis ; Lung disease ; Scoring system
    Abstract: Magnetic resonance imaging (MRI) gains increasing importance in the assessment of cystic fibrosis (CF) lung disease. The aim of this study was to develop a morpho-functional MR-scoring-system and to evaluate its intra- and inter-observer reproducibility and clinical practicability to monitor CF lung disease over a broad severity range from infancy to adulthood. 35 CF patients with broad age range (mean 15.3years; range 0.5-42) were examined by morphological and functional MRI. Lobe based analysis was performed for parameters bronchiectasis/bronchial-wall-thickening, mucus plugging, abscesses/sacculations, consolidations, special findings and perfusion defects. The maximum global score was 72. Two experienced radiologists scored the images at two time points (interval 10weeks). Upper and lower limits of agreement, concordance correlation coefficients (CCC), total deviation index and coverage probability were calculated for global, morphology, function, component and lobar scores. Global scores ranged from 6 to 47. Intra- and inter-reader agreement for global scores were good (CCC: 0.98 (R1), 0.94 (R2), 0.97 (R1/R2)) and were comparable between high and low scores. Our results indicate that the proposed morpho-functional MR-scoring-system is reproducible and applicable for semi-quantitative evaluation of a large spectrum of CF lung disease severity. This scoring-system can be applied for the routine assessment of CF lung disease and maybe as endpoint for clinical trials.
    Type of Publication: Journal article published
    PubMed ID: 21429685
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  • 4
    Keywords: Germany ; LUNG ; PERFUSION ; THERAPY ; CT ; imaging ; PATIENT ; MRI ; SEQUENCE ; MAGNETIC-RESONANCE ; magnetic resonance imaging ; AGE ; STATISTICAL-ANALYSIS ; MORPHOLOGY ; PULMONARY PERFUSION ; BODY ; CHILDREN ; SEGMENTS ; FEASIBILITY ; BREATH-HOLD ; LUNG PERFUSION ; fibrosis ; WEIGHT ; IMPAIRMENT ; CYSTIC-FIBROSIS ; cystic fibrosis ; SMALL AIRWAYS ; DEFECT ; GRAPPA ; CIRCULATION ; lung morphology
    Abstract: This paper is a feasibility study of magnetic resonance imaging (MRI) of lung perfusion in children with cystic fibrosis (CF) using contrast-enhanced 3D MRI. Correlation assessment of perfusion changes with structural abnormalities. Eleven CF patients (9 f, 2 m; median age 16 years) were examined at 1.5 T. Morphology: HASTE coronal, transversal (TR/TE/alpha/ST: 600 ms/28 ms/180 degrees/6 mm), breath-hold 18 s. Perfusion: Time-resolved 3D GRE pulse sequence (FLASH, TE/TR/alpha: 0.8/1.9 ms/40 degrees), parallel imaging (GRAPPA, PAT 2). Twenty-five data sets were acquired after intravenous injection of 0.1 mmol/kg body weight of gadodiamide, 3-5 ml/s. A total of 198 lung segments were analyzed by two radiologists in consensus and scored for morphological and perfusion changes. Statistical analysis was performed by Mantel-Haenszel chi-square test. Results showed that perfusion defects were observed in all patients and present in 80% of upper, and 39% of lower lobes. Normal lung parenchyma showed homogeneous perfusion (86%, P 〈 0.0001). Severe morphological changes led to perfusion defects (97%, P 〈 0.0001). Segments with moderate morphological changes showed normal (53%) or impaired perfusion (47%). In conclusion, pulmonary perfusion is easy to judge in segments with normal parenchyma or severe changes. In moderately damaged segments, MRI of lung perfusion may help to better assess actual functional impairment. Contrast-enhanced 3D MRI of lung perfusion has the potential for early vascular functional assessment and therapy control in CF patients
    Type of Publication: Journal article published
    PubMed ID: 16673092
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  • 5
    Keywords: Germany ; LUNG ; chest ; CT ; DIAGNOSIS ; FOLLOW-UP ; imaging ; DISEASE ; EXPOSURE ; RESOLUTION ; radiation ; PATIENT ; IMPACT ; prognosis ; MRI ; MAGNETIC-RESONANCE ; magnetic resonance imaging ; MORPHOLOGY ; COMPUTED-TOMOGRAPHY ; FUNCTION TESTS ; magnetic resonance imaging (MRI) ; CHILDREN ; HRCT ; fibrosis ; ADULTS ; LIFE ; CYSTIC-FIBROSIS ; TESTS ; technique ; function ; cystic fibrosis ; RADIATION EXPOSURE ; lungs ; improvement of ; gold ; mucoviscidosis
    Abstract: Cystic fibrosis (CF) is a multi-systemic disease with major impact on the lungs. Pulmonary manifestation is crucial for the prognosis and life expectancy of patients. Imaging modalities and lung function tests reflect the pulmonary status in these patients. The standard imaging modality for diagnosis and follow-up of pulmonary changes is chest x-ray. The gold standard for the detection of parenchymal lung changes remains high resolution computed tomography (HRCT), but this is not used routinely for CF-patients due to radiation exposure. Magnetic resonance imaging (MRI) used to be of no importance in monitoring cystic fibrosis lung disease, as shown in studies from the 1980s and early 1990s. The continuing improvement of MRI techniques, however, has allowed for an adequate application of this non-radiation method in diagnosing the major pulmonary findings in CF, in addition to the assessment of lung function
    Type of Publication: Journal article published
    PubMed ID: 16437239
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  • 6
    Keywords: BLOOD ; Germany ; LUNG ; SPIRAL CT ; VOLUME ; DISEASE ; POPULATION ; HEART ; TIME ; PATIENT ; BLOOD-FLOW ; blood flow ; FLOW ; MRI ; MAGNETIC-RESONANCE ; magnetic resonance imaging ; AGE ; arteries ; PARAMETERS ; HYPERTENSION ; HEALTHY ; PULMONARY ; VELOCITY ; fibrosis ; PH ; HEALTHY-VOLUNTEERS ; CHRONIC THROMBOEMBOLISM ; CYSTIC-FIBROSIS ; ARTERIAL ; PULMONARY-ARTERIES ; early development ; bronchosystemic shunt ; cystic fibrosis
    Abstract: Cystic fibrosis (CF) leads to disabling lung disease and pulmonary hypertension (PH). The goal of this study was to assess the hemodynamics in the systemic and pulmonary arterial circulation of patients with CF using MRI. Ten patients with CF and 15 healthy volunteers were examined (1.5-T MRI). Phase-contrast flow measurements were assessed in the ascending aorta, pulmonary trunc, and the left and right pulmonary arteries (PA), resulting in the following parameters: peak velocity (PV) (centimeters per second) velocity rise gradient (VRG), time to PV (milliseconds), and the average area (centimeters squared). The blood flow ratio between the right and left lungs and the bronchosystemic shunt were calculated. For the ascending aorta and pulmonary trunc no parameter was significantly different between both populations. In the right PA a significantly lower PV (p=0.001) and VRG (p=0.02) was found. In the left PA there was a significantly (p=0.007) lower PV but no significant (p=0.07) difference between the VRG. The areas of the right (p=0.08) and left (p=0.5) PA were not significantly enlarged. For the volunteers a linear increase of PV in both PA was found with age, while it decreased in patients with CF. The blood flow distribution (right/left lung) showed no significant (p=0.7) difference between the groups. There was a significantly (p 〈 0.001) higher bronchosystemic shunt volume in patients with CF (1.3 l/min) than in volunteers (0.1 l/min). Magnetic resonance based flow measurements in the right and left PA showed first indications for early development of PH. The significant increase in bronchosystemic shunt volume might be indicative fo the extent of parenchymal changes
    Type of Publication: Journal article published
    PubMed ID: 15761712
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  • 7
    Keywords: Germany ; LUNG ; chest ; CT ; FOLLOW-UP ; imaging ; INFORMATION ; SYSTEM ; TOOL ; DEATH ; POPULATION ; computed tomography ; RESOLUTION ; PATIENT ; IMPACT ; CONTRAST ; MRI ; MAGNETIC-RESONANCE ; magnetic resonance imaging ; COMPUTED-TOMOGRAPHY ; CHILDREN ; LUNG PERFUSION ; ADULTS ; PHASE ; cystic fibrosis
    Abstract: Cystic fibrosis (CF) is the most frequent inherited disorder leading to premature death in the Caucasian population. As life expectancy is limited by pulmonary complications, repeated imaging [chest X-ray, multislice high-resolution computed tomography (MS-HRCT)] is required in the follow-up. Magnetic resonance imaging (MRI) of the lung parenchyma is a promising new diagnostic tool. Its value for imaging lung changes caused by CF compared with CT is demonstrated. MRI performs well when compared with CT, which serves as the gold standard. Its lack in spatial resolution is obvious, but advantages in contrast and functional assessment compensate for this limitation. Thus, MRI is a reasonable alternative for imaging the CF lung and should be introduced as a radiation-free modality for follow-up studies in CF patients. For further evaluation of the impact of MRI, systematic studies comparing MRI and conventional imaging modalities are necessary. Furthermore, the value of the additional functional MRI (fMRI) information has to be studied, and a scoring system for the morphological and functional aspect of MRI has to be established
    Type of Publication: Journal article published
    PubMed ID: 16941092
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  • 8
    Keywords: evaluation ; Germany ; LUNG ; chest ; CT ; FOLLOW-UP ; imaging ; DISEASE ; EXPOSURE ; computed tomography ; NUCLEAR-MEDICINE ; radiation ; PATIENT ; MRI ; magnetic resonance ; MAGNETIC-RESONANCE ; magnetic resonance imaging ; AGE ; DAMAGE ; tomography ; COMPUTED-TOMOGRAPHY ; CHILDREN ; nuclear medicine ; COMPLICATIONS ; LUNG PERFUSION ; radiology ; ADULTS ; LIFE ; CHEST-X-RAY ; CYSTIC-FIBROSIS ; RADIATION-EXPOSURE ; methods ; NUCLEAR ; USA ; correlation ; cystic fibrosis ; female ; Male ; MEDICINE ; multidetector computed tomography ; - ; comparison ; RESONANCE ; MDCT ; chest x-ray ; cystic fibrosis (CF) ; morphologic MRI ; RADIOGRAPH ; SCORING SYSTEMS
    Abstract: Objectives: As pulmonary complications are life limiting in patients with cystic fibrosis (CF), repeated chest imaging [chest x-ray, computed tomography (CT)] is needed for follow up. With the continuously rising life expectancy of CF patients, magnetic resonance imaging (MRI) as a radiation-free imaging modality might become more and more attractive. The goal of this study was to prospectively assess the value of MRI for evaluation of morphologic pulmonary CF-changes in comparison to established imaging modalities. Materials and Methods: Thirty-one CF patients (19 female, 12 male; mean age 16.7 years) with stable lung disease were examined by MRI: HASTE, coronal/transversal (TR/TE/alpha/TA: infinite/28 ms/180 degrees/18 s), multi-detector computed tomography (MDCT) (30 patients): 120 kV, dose modulated mAs, and chest x-ray (21 patients). Image evaluation: random order, 4 chest radiologists in consensus; chest x-ray: modified Chrispin-Norman score; CT and MRI: modified Helbich score. The maximal attainable score for chest x-ray was 34, for MRI and CT 25. Median scores, Pearson correlation coefficients, Bland-Altman plots, and concordance of MRI to CT on a lobar and segmental basis were calculated. Results: The median MRI and MDCT scores were 13 (min 3, max 20) respectively 13.5 (min 0, max 20). The median chest x-ray score was 14 (min 5, max 32). Pearson correlation coefficients: MRI/CT = 0.80, P 〈 0.0001; MRI/chest x-ray = 0.63, P 〈 0.0018; chest x-ray/CT = 0.75, P 〈 0.0001. The median lobe related concordance was 80% for bronchiectasis, 77% for mucus plugging, 93%, for sacculation/abscesses, and 100% for collapse/consolidation. Conclusions: Morphologic MRI of the lung in CF patients demonstrates comparable results to MDCT and chest x-ray. Because radiation exposure is an issue in CF patients, MRI might have the ability to be used as an appropriate alternative method for pulmonary imaging
    Type of Publication: Journal article published
    PubMed ID: 17984769
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