From registry to quality management: the German Cystic Fibrosis Quality Assessment project 1995–2006

Stern, Martin; Wiedemann, Bärbel; Wenzlaff, Paul

doi:10.1183/09031936.00056507

Cited by 81 publications

(79 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Therefore, it is considerably difficult to predict the prognosis. In a retrospective study performed by Kerem et al (13) in 1992, it was reported that the two-year mortality was above 50%, if FEV1-expected was ≤30%, PaO 2 was <55 n (%) Chronic (14,15,16,17,18,19,20,21,22). In our study, a FEV1-expected % value of ≤30 was related with increased mortality risk and 75% of these patients were lost in an average period of 2.5 years.…”

Section: Discussionmentioning

confidence: 50%

“…In this study, the most valuable variable in determining the prognosis was found to be FEV1 percent. Following this study many studies have been performed to determine the mortality risk in patients with cystic fibrosis (14,15,16,17,18,19,20,21,22). The aim of these studies was to make a right selection for the patients who needed lung transplantation, because even if transplantation is performed, there is a morbidity and mortality risk related to early and late complications (22).…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Orta ve ağır akciğer tutulumu olan kistik fibrozlu hastalarda ölüme etki eden diğer risk etmenleri

...

2012

Tpa

View full text Add to dashboard Cite

Aim:Severe pulmonary disease is responsible for over 80% of deaths associated with cystic fibrosis. Our aim was to evaluate the other risk factors associated with mortality in patients with cystic fibrosis who have moderate and severe lung disease. Material and Method: Among 200 patients with cystic fibrosis who were followed up in our clinic, 35 patients with moderate and severe lung disease (%FEV1 predicted ≤60) were included in the study. Demographic data, pulmonary function tests, high resolution thorax tomography, modified Bhalla score, blood gas analysis and nutritional status of the patients were evaluated. Results: Requirement for respiratory support was 31.4% in moderate and severe lung disease. Lower pulmonary function, requirement for non invasive ventilation, presence of diabetes mellitus, a Sa0 2 value of <95, a value of pCO 2 >50 mm Hg and requirement for frequent IV antibiotics were significantly related with increased mortality. Conclusions: This is a single centre study conducted in patients with cystic fibrosis who had moderate and severe lung disease and who had not received lung transplantation. We evaluated the clinical outcome and other factors that were associated with mortality. (Turk Arch Ped 2012; 47: 263-267)

show abstract

Section: Discussionmentioning

confidence: 50%

Section: Introductionmentioning

confidence: 99%

Orta ve ağır akciğer tutulumu olan kistik fibrozlu hastalarda ölüme etki eden diğer risk etmenleri

...

2012

Tpa

View full text Add to dashboard Cite

show abstract

“…These differences persisted after taking into account socio-economic factors. Similar features can be derived from the CF German Registry where a quality management program with an overall coverage of 82% for the year of 2005 confirmed considerable differences between centres in terms of key parameters (Stern et al, 2008). For instance, the percentage of children (6-18 years) with an FEV 1 above 80% of the predicted value ranged from 20 to 100% in centres treating less than 50 patients and from 35% to 100% in larger centres.…”

Section: Cf Centres Might Not Be Sufficientmentioning

confidence: 55%

The Prognosis of Cystic Fibrosis - A Clinician's Perspective

Lebecque¹

2012

Cystic Fibrosis - Renewed Hopes Through Research

View full text Add to dashboard Cite

“…[2][3][4] Owing to advances in management and treatment of these pulmonary manifestations, the life expectancy of patients is continuously increasing, with a current median survival of approximately 40 years. [3][4][5] Accurate diagnosis and monitoring of CF-related lung disease is crucial in providing early and effective treatment and also in improving the overall prognosis. Thin-sliced chest computed tomography (CT) is the current criterion standard to visualize best the characteristic morphologic pulmonary changes due to its high spatial resolution.…”

Section: Discussionmentioning

confidence: 99%

“…Results: The overall modified Helbich CT score had a mean (SD) of 15. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] and median of 15.0 (IQR, 7.3). Without assessment of perfusion, the overall Eichinger score resulted in the following values for CT vs MR examinations: mean (SD), 20.3 (7.2) (range, 4-31); and median, 21.0 (IQR, 9.5) vs mean (SD), 19.5 (7.1) (range, ; and median, 20.0 (IQR, 9.0).…”

mentioning

confidence: 99%

Comparison Between Magnetic Resonance Imaging and Computed Tomography of the Lung in Patients With Cystic Fibrosis With Regard to Clinical, Laboratory, and Pulmonary Functional Parameters

Renz¹,

Scholz

Böttcher

et al. 2015

Investigative Radiology

View full text Add to dashboard Cite

Objective: To evaluate whether magnetic resonance imaging (MRI) is effective as computed tomography (CT) in determining morphologic and functional pulmonary changes in patients with cystic fibrosis (CF) in association with multiple clinical parameters. Materials and Methods: Institutional review board approval and patient written informed consent were obtained. In this prospective study, 30 patients with CF (17 men and 13 women; mean (SD) age, 30.2 (9.2) years; range, 19-52 years) were included. Chest CT was acquired by unenhanced low-dose technique for clinical purposes. Lung MRI (1.5 T) comprised T2-and T1-weighted sequences before and after the application of 0.1-mmol·kg −1 gadobutrol, also considering lung perfusion imaging. All CT and MR images were visually evaluated by using 2 different scoring systems: the modified Helbich and the Eichinger scores. Signal intensity of the peribronchial walls and detected mucus on T2-weighted images as well as signal enhancement of the peribronchial walls on contrast-enhanced T1-weighted sequences were additionally assessed on MRI. For the clinical evaluation, the pulmonary exacerbation rate, laboratory, and pulmonary functional parameters were determined. .2) (range, 4-31); and median, 21.0 (IQR, 9.5) vs mean (SD), 19.5 (7.1) (range, 4-33); and median, 20.0 (IQR, 9.0). All differences between CT and MR examinations were not significant (Wilcoxon rank sum tests and Student t tests; P > 0.05). In general, the correlations of the CT scores (overall and different imaging parameters) to the clinical parameters were slightly higher compared to the MRI scores. However, if all additional MRI parameters were integrated into the scoring systems, the correlations reached the values of the CT scores. The overall image quality was significantly higher for the CT examinations compared to the MRI sequences. Conclusions:One major diagnostic benefit of lung MRI in CF is the possible acquisition of several different morphologic and functional imaging features without the use of any radiation exposure. Lung MRI shows reliable associations with CT and clinical parameters, which suggests its implementation in CF for routine diagnosis, which would be particularly important in follow-up imaging over the long term.

show abstract

From registry to quality management: the German Cystic Fibrosis Quality Assessment project 1995–2006

Cited by 81 publications

References 27 publications

Orta ve ağır akciğer tutulumu olan kistik fibrozlu hastalarda ölüme etki eden diğer risk etmenleri

Orta ve ağır akciğer tutulumu olan kistik fibrozlu hastalarda ölüme etki eden diğer risk etmenleri

The Prognosis of Cystic Fibrosis - A Clinician's Perspective

Comparison Between Magnetic Resonance Imaging and Computed Tomography of the Lung in Patients With Cystic Fibrosis With Regard to Clinical, Laboratory, and Pulmonary Functional Parameters

Contact Info

Product

Resources

About