WS3.6 The CF-ABLE score: a novel clinical prediction tool in cystic fibrosis

McCarthy, Cormac; Dimitrov, Borislav D.; Gunaratnam, C.; McElvaney, N.G.

doi:10.1016/s1569-1993(12)60024-1

Cited by 15 publications

(29 citation statements)

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“…2,5,6 McCarthy et al used data from the Irish CF Registry to validate a prediction tool for CF prognosis (CF-ABLE-score). 10 The score was created combining age, BMI, FEV 1 , and number of exacerbations (PEx) in the last 3 months; lower scores were associated with lower risk of death or lung transplantation in the next 4 years. A limitation of the study is the small number of patients (49) in the derivation sample (370 patients for validation).…”

Section: Mortality and Survivalmentioning

confidence: 99%

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Salvatore

Buzzetti

Mastella

2016

Pediatric Pulmonology

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Patient registries provide useful information to afford more knowledge on rare diseases like Cystic Fibrosis (CF). Twenty-two studies originating from national CF registries, focusing on demographics, survival, genetics, nutritional status, and non-pulmonary complications, were published between December 2011 and March 2015. The purpose of this review article is to examine these reports, aiming attention to the clinical characteristics of CF patients included in the registries, current, and estimated future epidemiological data, the role of gender gap, the increasing survival in different countries. Some studies offer insights into pubertal growth and non-pulmonary complications, such as liver disease, nephropathy, and cancer. Pediatr Pulmonol. 2017;52:390-398. © 2016 Wiley Periodicals, Inc.

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Section: Mortality and Survivalmentioning

confidence: 99%

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Salvatore

Buzzetti

Mastella

2016

Pediatric Pulmonology

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“…The impact of malnutrition in CF is of great interest; exocrine and endocrine dysfunction, coupled with high basal metabolic requirements in the setting of chronic disease, exposes patients to a high risk of malnourishment. Low BMI in CF is known to increase in incidence with age and be closely implicated in worsening lung function [91,92] and predicts worse outcome over a 4-year period [93]. Furthermore, patients awaiting lung transplantation who require nutritional intervention have been shown to have a higher risk of death [86] and improving the nutritional status of malnourished patients may ultimately improve lung function, even in cohorts with relatively advanced lung disease [94][95][96].…”

Section: Nutritional Status and Weight In Determining Outcome In Cystmentioning

confidence: 99%

“…Biomarkers specific to pulmonary exacerbations are ideal as defining the response to treatment is very difficult in CF,; these include mucins MUC5AB and MUC5AC which are degraded at the time of exacerbation and demonstrate increased sialylation compared to controls [114]. Granulocyte-macrophage colony-stimulating factor (GM-CSF) levels at the time of pulmonary exacerbation predicts a larger acute decline in FEV1 [115], which is highly predictive of poor prognosis [13], hence this may be a useful marker of future disease progression. Finally, calprotectin, a neutrophil derived protein, may also be a useful biomarker, where levels both in sputum and serum have been demonstrated to decrease significantly following treatment of an acute exacerbation and predicted time to next exacerbation [116], this is extremely useful as both exacerbations and lung function decline are the most sensitive markers of disease progression in CF.…”

Section: Sputum and Bronchoalveolar Lavage Fluid Biomarkersmentioning

confidence: 99%

“…However, it remains a complicated score to perform clinically and so focus has shifted in recent times to the development of simplified prediction tools that can be used within the time constraints of the current clinical setting. The CF-ABLE score was devised with this in mind and it involves the measurement of 4 of the major clinical parameters encountered in day to day practice [93]. Age, BMI, FEV1, and frequency of pulmonary exacerbations have been evaluated on a 7-point scale for correlation with prognosis and it has been found that patients with higher scores have a 26% chance of a poor outcome (death or transplantation) within 4 years of measurement.…”

Section: Usefulness Of Clinical Prediction Tools In Cystic Fibrosismentioning

confidence: 99%

“…Moreover, there has been huge progress in the research into and development of biomarkers of inflammation in CF [10][11][12], both systemic and pulmonary inflammation that correlates with clinical condition and can predict outcome, further highlighting the deeper understating of the pathophysiological changes in CF and the translational research ongoing in this area. Furthermore, the use of new composite scoring systems, taking into account many aspects of this multisystem condition have been developed to aid with the classification of disease severity and predict outcome over a defined period [13].…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Factors Affecting Prognosis and Prediction of Outcome in Cystic Fibrosis Lung Disease

McCarthy¹,

O’Carroll²,

Franciosi³

et al. 2015

Cystic Fibrosis in the Light of New Research

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Cystic fibrosis CF is a multisystem disorder with a significantly shortened life expectancy with the major cause of mortality related to lung disease. Inflammation is seen in the CF airways from a very early age and contributes significantly to symptoms and disease progression. "s the condition worsens over time, lung function declines, usually measured by Forced Expiratory Volume in second FEV % predicted, and extra-pulmonary complications often manifest. While the life expectancy in CF is still short, the median age of death and predicted survival age are continually increasing. Therapeutic interventions for CF have improved significantly in the last years and now there are targeted therapies towards specific elements in CF that may impact upon exacerbation frequency, symptoms, and eventually mortality due to lung disease.

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Sputum neutrophil elastase and its relation to pediatric bronchiectasis severity: A cross‐sectional study

Ali

Fouda

Salem

et al. 2022

Health Science Reports

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Background and Aims: Sputum neutrophil elastase (NE) is a marker of neutrophilic airway inflammation in bronchiectasis. Yet, not much is known about its role in pediatric bronchiectasis severity. This study aimed to assess the sputum NE value as a biomarker of clinical and radiological severity in pediatric bronchiectasis.Methods: This was a cross-sectional study assessing sputum NE in a total of 50 bronchiectasis patients under the age of 18 years-30 patients with cystic fibrosis (CF) and 20 patients with non-CF bronchiectasis were included. Bronchiectasis severity was assessed using Shwachman-Kulczycki (SK) score, CF-ABLE score, and CF risk of disease progression score, among CF patients, and bronchiectasis severity index (BSI) and FACED criteria among non-CF bronchiectasis patients, associations between sputum NE and bronchiectasis severity were assessed in both patient groups.Results: Sputum NE was directly correlated with C-reactive protein (r = 0.914, p < 0.001), (r = 0.786, p < 0.001), frequency of exacerbations (r = 0.852, p < 0.001) (r = 0.858, p < 0.001), exacerbations severity (r = 0.735, p = 0.002), (r = 0.907, p < 0.001), and the number of hospital admissions (r = 0.813, p < 0.001), (r = 0.612, p =0.004) in the last year among CF, and non-CF bronchiectasis patients, respectively. Additional linear correlations were found between sputum NE, CF risk of disease progression score (p < 0.001), CF-ABLE score (p < 0.001), and lower forced expiratory volume 1% of predicted (p = 0.017; ρ = −0.8) among CF patients. Moreover, sputum NE was positively correlated with the neutrophil count (p = 0.018), and BSI severity score (p = 0.039; ρ = 0.465) among non-CF bronchiectasis patients.Conclusions: Sputum NE may be considered a good biomarker of bronchiectasis severity in both CF and non-CF bronchiectasis patients, as confirmed by the exacerbations rate, CF risk of disease progression, and BSI scores.

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WS3.6 The CF-ABLE score: a novel clinical prediction tool in cystic fibrosis

Cited by 15 publications

References 0 publications

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Factors Affecting Prognosis and Prediction of Outcome in Cystic Fibrosis Lung Disease

Sputum neutrophil elastase and its relation to pediatric bronchiectasis severity: A cross‐sectional study

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