Tag Archives: Cystic Fibrosis

Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: Recommendation

Our findings would support the concept that while longitudinal structural abnormalities are clearly seen with HRCT, their longitudinal functional significance is less clear-cut. Other imaging techniques such as hyperpolarized He MRI may prove a useful alternative in obtaining longitudinal functional information. We recently evaluated He MRI in CF adults and found it to be a strong correlate of structural HRCT abnormalities and a stronger correlate of spirometry than HRCT. We envisage, although currently hypothetical, that spirometry, He MRI, and HRCT may work synergistically to provide longitudinal functional and structural information in CF. add comment
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Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: Conclusion

Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: ConclusionClearly there were differences between the Irish and Swedish cohorts in longitudinal progression, with the annual change in CT score equal to + 1.6% and FEV1 equal to — 0.4%, compared to – 2.7% and -2.3% in the Irish cohort, respectively. For individual HRCT abnormalities, de Jong et al found no change in mucus plugging over time in their adult patients in contrast to our results in which mucus plugging worsened predominantly in the group with mildly impaired lung function. Such discrepancies may be related to differences in treatment or in patient populations. Some European centers have followed a more aggressive policy of early antibiotic intervention from time of diagnosis, which may influence decline in spirometry results over time. More info

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Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: Discussion

Severity of bronchiectasis, extent of mucus plugging, sacculations/abscesses, generations of bronchial divisions involved, and air trapping demonstrated systematic differences between readers on Bland and Altman plots. Observer 1 scored extent of mucus plugging and air trapping worse on average than observer 2, and this was more pronounced for mild scores. Observer 2 scored severity of bronchiectasis, sacculations/abscesses, and generations of bronchial divisions involved worse on average than observer 1. For severity of bronchiectasis and generations of bronchial divisions involved, this was more pronounced for moderate scores; for sacculations/abscesses, this was more pronounced for severe scores. Reading here
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Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: HRCT Findings for All Patients

Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: HRCT Findings for All PatientsOn an annual basis, all spirometric variables except RV deteriorated significantly (Table 4). Almost all individual HRCT parameters also deteriorated, with the exception of acinar nodules, mosaic perfusion, and thickening of interlobular septa (Fig 1, bottom, B). Air trapping, collapse/consolidation, mucus plugging, peribronchial thickening, severity and extent of bronchiectasis, generations of bronchial divisions involved, and HRCT total score declined significantly faster than FEV1. FEF25-75 declined at a rate of – 5.1% per year. However, air trapping and extent of bronchiectasis declined significantly faster, at – 5.3 per year and – 6.5% per year, respectively (p < 0.001). Six patients showed stable spirometry results (annual change, 1.4% per year) but worsening HRCT scores (annual change, 6.6% per year). Reading here
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Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: Results

Mean age of the patients increased by 4 years (Table 2). Mean BMI remained stable, but four patients had BMIs < 18.5 kg/m2 at time 1, indicating malnourishment. Genotype was available for 37 patients. Forty-four percent of patients were homozygous and 36% were heterozygous for the AF508 mutation. All spirometry measurements except RV deteriorated significantly. Mean FEV1 was 81.8 ± 24.9% of predicted at time 1, indicating a group with overall mild lung impairment (Fig 1, top, A). This decreased by 11.7% between PFT-1 and PFT-2 (42 ± 7 months). Sixteen patients had mild lung function abnormalities, with mean FEV1 decreasing from 102.1 ± 14.5 to 87.5 ± 18.2%. Nineteen patients had moderate lung function abnormalities, with mean FEV1 decreasing from 69.8 ± 6.0 to 59.5 ± 15.8%. Four patients had severe lung function abnormalities, with mean FEV1 decreasing from 34.0 ± 2.7 to 30.5 ± 2.6%.

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Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: Longitudinal Measurements and Statistical Calculations

Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: Longitudinal Measurements and Statistical CalculationsThe initial HRCT and spirometry findings obtained at time 1 are reported as HRCT-1 and PFT-1, and follow-up HRCT and spirometry findings obtained at time 2 are reported as HRCT-2 and PFT-2. Median time interval was 42 ± 16 months (range, 19 to 73 months). The annual rate of change in HRCT score (AHRCT) was calculated for each patient as follows: (HRCT-2 — HRCT-1)/individual time interval for each patient. A positive AHRCT value indicated a worsening in HRCT appearances. Similarly, the annual rate of change in PFT score (APFT) was calculated for each patient as follows: (PFT-2 — PFT-1)/individ-ual time interval for each patient. A negative APFT value indicated a worsening in spirometry results. http://www.medicines-for-diabetes.com/
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Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: Sputum

Areas of hyperlucency with hypovascularity defined mosaic perfusion on inspiratory scans. Areas of hyperlucency on expiratory images defined air trapping. The total score was derived by adding the scores for each abnormality, and ranged from 0 to 37. For scoring purposes, the average of the two observers’ individual scores were calculated. Absolute total scores were converted to percentages of the potential total maximum score. The extent and severity of abnormalities were scored as in Table 1.
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Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: HRCT Protocol and Scoring

Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: HRCT Protocol and ScoringOf the two observers, one observer (J.M.) was a senior chest radiologist with > 20 years experience. The other observer (J.D.) was a thoracic clinical fellow in chest radiology with 2 years experience scoring HRCT. A consensus meeting was held prior to formal scoring to standardize scoring between observers. HRCT scans were scored over a 2-week time period.
Images were scored using a modified Bhalla scoring system. Modifications included additional HRCT abnormalities described in CF lung disease since the original publication by Bhalla et al. Abnormalities were defined according to recommendations of the nomenclature committee of the Fleischner society. Bronchiectasis was defined as a bronchus with an internal diameter larger then its accompanying pulmonary artery, lack of tapering of the bronchial lumen for > 2 cm, and visualization of a bronchus within 1 cm of the costal pleura. Peribronchial thickening was defined by a bronchial wall > 1 mm in thickness. Mucus plugging was identified by visualizing plugs in large airways or by the presence of tree-in-bud peripherally.

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Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: Patient Population

The CF radiology database was retrospectively searched from January 1997 to January 2005 by a single investigator (E.J.) for 39 consecutive patients with two HRCT scans (HRCT-1 and HRCT-2) > 18 months apart (19 males and 20 females; mean age, 22 years; range, 16 to 48 years). All patients had documented clinical, radiologic, or genotypic features of CF as well as abnormal sweat test results (sweat sodium and chloride > 60 mmol/L).
Clinical charts were reviewed and age, body mass index (BMI), spirometry, and sputum cultures were recorded at the time of the two HRCT scans. Only clinically stable patients at the time of a HRCT scan were included. Patients were excluded if they had the following: (1) only one HRCT (or two HRCTs < 18 months apart); (2) symptoms or signs of acute respiratory exacerbation at the time of HRCT or spirometry; (3) unstable spirometry results at the time of either HRCT (> 10% decrease in FEV1 compared with baseline values in the preceding 2 months); (4) required hospitalization for IV antibiotics in the 2 weeks prior to either HRCT for a respiratory tract infection; and (5) died before undergoing sequential HRCT. This resulted in approximately 210 patients being excluded from the study, leaving 39 in the final study group. The hospital ethics committee of out institution approved the study.
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Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis

Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic FibrosisCystic fibrosis (CF) is the most common autosomal recessive inherited disorder in whites, with a prevalence of 1 in 1,461 births in the Irish population. By the later teenage years, significant irreversible lung damage has occurred in many patients. The majority die of recurrent respiratory sepsis and respiratory failure in adulthood.
Spirometry, particularly FEV1, is used as a marker of lung disease because of its reproducibility and accuracy as an outcome surrogate. It is well known that spirometry results decline as lung disease progresses. In a thought-provoking article, de Jong et al showed that the rate of decline in high-resolution CT (HRCT) appearances was more significant than the rate of decline in spirometry results in children, and this has been confirmed by others. More recently, further novel work has shown that several HRCT abnormalities decline at a faster rate than FEV1 in adults. As a result, proposals for the use of HRCT as an outcome surrogate have been suggested.” In this regard, a combined HRCT/ spirometric score has been shown to be a sensitive marker in detecting treatment effects. More recently, HRCT has been shown to correlate with the number of respiratory exacerbations in children over time.
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