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

McCarthy, Cormac; O’Carroll, Orla; Franciosi, Alessandro N; McElvaney, NoelG.

doi:10.5772/60899

Cited by 5 publications

(7 citation statements)

References 156 publications

(172 reference statements)

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“…Low BMI in CF is known to be closely implicated in worsening lung function and predicts worse outcome over a 4-year period. 18 Our results support the importance of an adequate nutritional status and optimal airflow patency for preserving the ability to generate an adequate working rate, and…”

Section: Discussionsupporting

confidence: 77%

“…Indeed malnutrition can lead to the loss of body fat and free fat mass, with muscle mass as the main part of it. Low BMI in CF is known to be closely implicated in worsening lung function and predicts worse outcome over a 4‐year period . Our results support the importance of an adequate nutritional status and optimal airflow patency for preserving the ability to generate an adequate working rate, and show also that chronic infection by P. aeruginosa represents a limiting factor.…”

Section: Discussionsupporting

confidence: 77%

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Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross‐sectional study

Gambazza

Guarise

Carta

et al. 2020

Pediatric Pulmonology

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Background Lung clearance index (LCI2.5) is a marker of overall lung ventilation inhomogeneity and has proven to be able to detect early peripheral damage in subjects with cystic fibrosis (CF), with greater sensitivity than conventional spirometry. Combining its sensitivity with the output of an incremental exercise testing, we hypothesized that any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal exercise tolerance could be relevant for the CF team, tracking early lung disease and potential exercise limiting factors. Methods Patients with CF in clinical stable conditions were recruited between 2015 and 2017. Available spirometry, nitrogen multiple‐breath washout test and symptoms‐limited exercise testing performed as parts of patients' annual routine evaluation were considered for this cross‐sectional study. To describe the relationship between exercise intensity and ventilation inhomogeneity, a linear regression analysis was performed using backward elimination based on Akaike information criteria. Results Seventy‐seven patients (38 females) were included. Sacin and LCI2.5 were significantly higher in patients with an overall reduced exercise tolerance. Peak work developed during exercise was associated with body mass index (b = 5.25; 95% confidence interval [CI] = 1.53‐8.98), forced expiratory volume in 1 second (FEV1; b = 3.71; 95% CI = 1.96‐5.46), Pseudomonas aeruginosa chronic infection (b = −8.84; 95% CI = −15.84 to −1.84) but not with LCI2.5. Conclusion Exercise capacity and airflow obstruction are associated in this Italian CF cohort. Considering the greater discriminatory power of LCI2.5 over FEV1 and peak work, the Godfrey protocol without gas analysis cannot provide detailed information about lung function or efficiency. However, this incremental protocol without gas exchange measures can still provide the CF team with information about exercise tolerance and disability.

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Section: Discussionsupporting

confidence: 77%

Section: Discussionsupporting

confidence: 77%

Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross‐sectional study

Gambazza

Guarise

Carta

et al. 2020

Pediatric Pulmonology

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“…As discussed above, classical sputum and bronchoalveolar lavage fluid (BALF) prognostic biomarkers of CF include elevated levels of IL-8 chemokine and other cytokines, such as IL-10, IL-4, and TNF-alpha, as well as increased neutrophil elastase (NE) or myeloperoxidase (MPO) activity [ 2 , 4 , 10 , 11 , 13 , 43 , 62 , 63 ]. Elevated levels of NFkB and metalloproteinases (MMP), namely MMP-2 and MMP-9, are associated with increased NE activity and CF lung disease pathogenesis and progression [ 11 , 42 , 64 ].…”

Section: Prognostic Biomarkers Of Cystic Fibrosis Lung Diseasementioning

confidence: 99%

“…Moreover, serum IL-8 and TNF-α levels are similarly elevated during exacerbation as well as constitutively, as compared with healthy or non-CF subjects. C-reactive protein (CRP), serum amyloid A, leukocyte RNA, and AAT:CD16b complex [ 11 , 43 , 44 , 45 ] are other novel biomarkers that have been evaluated for CF prognosis. NPD, PFT, and sweat chloride remain gold standard outcome measure of therapeutic efficacy and/or disease progression; however, the effectiveness of currently available biomarkers is associated with one or more CF outcomes with low effect sizes, making them unacceptable for clinical application [ 46 ] or predicting disease or its severity.…”

Section: Introductionmentioning

confidence: 99%

Prognosis-Based Early Intervention Strategies to Resolve Exacerbation and Progressive Lung Function Decline in Cystic Fibrosis

Vij

2021

JPM

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Cystic fibrosis (CF) is a genetic disease caused by a mutation(s) in the CF transmembrane regulator (CFTR), where progressive decline in lung function due to recurring exacerbations is a major cause of mortality. The initiation of chronic obstructive lung disease in CF involves inflammation and exacerbations, leading to mucus obstruction and lung function decline. Even though clinical management of CF lung disease has prolonged survival, exacerbation and age-related lung function decline remain a challenge for controlling the progressive lung disease. The key to the resolution of progressive lung disease is prognosis-based early therapeutic intervention; thus, the development of novel diagnostics and prognostic biomarkers for predicting exacerbation and lung function decline will allow optimal management of the lung disease. Hence, the development of real-time lung function diagnostics such as forced oscillation technique (FOT), impulse oscillometry system (IOS), and electrical impedance tomography (EIT), and novel prognosis-based intervention strategies for controlling the progression of chronic obstructive lung disease will fulfill a significant unmet need for CF patients. Early detection of CF lung inflammation and exacerbations with the timely resolution will not only prolong survival and reduce mortality but also improve quality of life while reducing significant health care costs due to recurring hospitalizations.

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“…Numerous factors are associated with prognosis, including genotype, sex and clinical measurements such as pulmonary function. Several tools for prediction of survival in CF have been developed: for summaries see Buzetti et al [5], McCarthy et al [6], MacNeill [7]. Data from national patient registries provide the potential to develop prediction tools using longitudinal information from large samples with long-term follow-up and such tools have been developed using CF patient registry data in the US [8], Canada [9] and France [10].…”

Section: Introductionmentioning

confidence: 99%

Results from an online survey of adults with cystic fibrosis: Accessing and using life expectancy information

et al. 2019

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Cystic fibrosis (CF) is the one of the most common inherited diseases. It affects around 10,000 people in the UK, and the median survival age is 47. Recent developments making use of longitudinal patient registry data are producing more detailed and relevant information about predicted life expectancy in CF based on current age and clinical measurements. The objective of this study was toconduct an online survey of adults with CF living in the UK using a web-based questionnaire to investigate: (i) if and how they access information on life expectancy; (ii) what they use it for; (iii) if they want more personalised information on life expectancy or the time until other milestones. The survey was advertised through the Cystic Fibrosis Trust using social media. There were 85 respondents, covering men (39%) and women (61%) aged 16–65. 75% had received information on life expectancy either from their CF care team (34%) or other sources (71%), the most common being the Cystic Fibrosis Trust website and research literature. Most people who received information found it to be beneficial and reported using it in a variety of ways, including to plan strategies for maintaining as best health as possible and to psychologically manage current health status. 82% of respondents were interested in more personalised information about their life expectancy, and participants also noted interest in other outcomes, including time to needing transplant or reaching a low level of lung function. Themes arising in text responses included the importance of good communication of information, the difficulty of relating general information to one’s own circumstances, and a desire for increased information on factors that impact on survival in CF. As an outcome from this work, research is underway to establish how information on life expectancy can be presented to people with CF in an accessible way.

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

Cited by 5 publications

References 156 publications

Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross‐sectional study

Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross‐sectional study

Prognosis-Based Early Intervention Strategies to Resolve Exacerbation and Progressive Lung Function Decline in Cystic Fibrosis

Results from an online survey of adults with cystic fibrosis: Accessing and using life expectancy information

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