Lung function in children with cystic fibrosis in the USA and UK: a comparative longitudinal analysis of national registry data

RationaleA previous analysis found significantly higher lung function in the US paediatric cystic fibrosis (CF) population compared with the UK with this difference apparently decreasing in adolescence and adulthood. However, the cross-sectional nature of the study makes it hard to interpret these results.ObjectivesTo compare longitudinal trajectories of lung function in children with CF between the USA and UK and to explore reasons for any differences.MethodsWe used mixed effects regression analysis to model lung function trajectories in the study populations. Using descriptive statistics, we compared early growth and nutrition (height, weight, body mass index), infections (Pseudomonas aeruginosa, Staphylococcus aureus) and treatments (rhDnase, hypertonic saline, inhaled antibiotics).ResultsWe included 9463 children from the USA and 3055 children from the UK with homozygous F508del genotype. Lung function was higher in the USA than in the UK when first measured at age six and remained higher throughout childhood. We did not find important differences in early growth and nutrition, or P.aeruginosa infection. Prescription of rhDNase and hypertonic saline was more common in the USA. Inhaled antibiotics were prescribed at similar levels in both countries, but Tobramycin was prescribed more in the USA and colistin in the UK. S. aureus infection was more common in the USA than the UK.ConclusionsChildren with CF and homozygous F508del genotype in the USA had better lung function than UK children. These differences do not appear to be explained by early growth or nutrition, but differences in the use of early treatments need further investigation.

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How do growth and nutrition explain social inequalities in lung function in children with cystic fibrosis? A longitudinal mediation analysis using interventional disparity effects with time-varying mediators and intermediate confounders.

10.1101/2022.01.11.22268909 ◽

2022 ◽

Author(s):

Daniela K Schlueter ◽

Ruth Keogh ◽

Rhian Daniel ◽

Schadrac Agbla ◽

David Taylor-Robinson

Keyword(s):

Cystic Fibrosis ◽

Lung Function ◽

Mediation Analysis ◽

Social Inequalities ◽

Early Growth ◽

Forced Expiratory Volume ◽

Time Varying ◽

Disadvantaged Children ◽

The Uk ◽

Weight Trajectories

Background Deprivation is associated with poorer growth, worse lung function and shorter life expectancy in children with cystic fibrosis (CF). While early growth is associated with lung function when first measured at around age 6, it is unclear whether improving early growth in the most disadvantaged children would reduce inequalities in lung function. Methods We used data from children born 2000-2010 and followed up to 2016 in the UK CF Registry. To estimate the association between deprivation and lung function at around age six, and the causal contribution of early weight trajectories, we extended the mediation analysis approach based on interventional disparity effects to the setting of a longitudinally measured mediator. We adjusted for baseline confounding by sex, birthyear and genotype and accounted for time-varying intermediate confounding by lung infection. Results 853 children were included in the study, including 165 and 172 children from the least and most deprived population quintiles, respectively. The average difference in lung function between the least and most deprived quintile of children, was 4.51 percent of predicted forced expiratory volume in one second (95% CI: 1.08-7.93). We estimated this would be reduced to 3.97 percentage points (95% CI: 0.57-7.38) if early weight trajectories in the most deprived children were shifted to the distribution observed in the least disadvantaged children. Conclusion Socio-economic conditions are strongly associated with lung function for children with CF which we estimated would only be marginally reduced if early weight trajectories could be improved for the most disadvantaged children.

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Faculty Opinions recommendation of Relationship between lung function and Modified Shuttle Test performance in adult patients with cystic fibrosis: a cross-sectional, retrospective study.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.726401929.793519517 ◽

2016 ◽

Author(s):

Shruti Paranjape

Keyword(s):

Cystic Fibrosis ◽

Retrospective Study ◽

Lung Function ◽

Test Performance ◽

Adult Patients ◽

Cross Sectional

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Relationship between lung function and Modified Shuttle Test performance in adult patients with cystic fibrosis: a cross-sectional, retrospective study

Physiotherapy ◽

10.1016/j.physio.2015.10.015 ◽

2016 ◽

Vol 102 (2) ◽

pp. 184-188 ◽

Cited By ~ 7

Author(s):

W.R. Doeleman ◽

T. Takken ◽

I. Bronsveld ◽

E.H.J. Hulzebos

Keyword(s):

Cystic Fibrosis ◽

Retrospective Study ◽

Lung Function ◽

Test Performance ◽

Adult Patients ◽

Cross Sectional

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Health at the ballot box: disease threat does not predict attractiveness preference in British politicians

Royal Society Open Science ◽

10.1098/rsos.160049 ◽

2016 ◽

Vol 3 (3) ◽

pp. 160049

Author(s):

Gustav Nilsonne ◽

Adam Renberg ◽

Sandra Tamm ◽

Mats Lekander

Keyword(s):

Infant Mortality ◽

Life Expectancy ◽

Good Health ◽

Cross Sectional Study ◽

Cross Sectional ◽

Self Rated Health ◽

Members Of Parliament ◽

The Usa ◽

Adaptive Behaviours ◽

The Uk

According to disease avoidance theory, selective pressures have shaped adaptive behaviours to avoid people who might transmit infections. Such behavioural immune defence strategies may have social and societal consequences. Attractiveness is perceived as a heuristic cue of good health, and the relative importance of attractiveness is predicted to increase during high disease threat. Here, we investigated whether politicians' attractiveness is more important for electoral success when disease threat is high, in an effort to replicate earlier findings from the USA. We performed a cross-sectional study of 484 members of the House of Commons from England and Wales. Publicly available sexiness ratings (median 5883 ratings/politician) were regressed on measures of disease burden, operationalized as infant mortality, life expectancy and self-rated health. Infant mortality in parliamentary constituencies did not significantly predict sexiness of elected members of parliament ( p = 0.08), nor did life expectancy ( p = 0.06), nor self-rated health ( p = 0.55). Subsample analyses failed to provide further support for the hypothesis. In conclusion, an attractive leader effect was not amplified by disease threat in the UK and these results did not replicate those of earlier studies from the USA concerning the relationship between attractiveness, disease threat and voting preference.

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Microbiological contamination of nebulizers used by cystic fibrosis patients: an underestimated problem

Jornal Brasileiro de Pneumologia ◽

10.1590/1806-3713/e20170351 ◽

2019 ◽

Vol 45 (3) ◽

Cited By ~ 9

Author(s):

Barbara Riquena ◽

Luciana de Freitas Velloso Monte ◽

Agnaldo José Lopes ◽

Luiz Vicente Ribeiro Ferreira da Silva-Filho ◽

Neiva Damaceno ◽

...

Keyword(s):

Cystic Fibrosis ◽

Burkholderia Cepacia ◽

Tap Water ◽

Significant Proportion ◽

Burkholderia Cepacia Complex ◽

Structured Interviews ◽

Candida Spp ◽

Cross Sectional ◽

Inhaled Antibiotics ◽

Cleaning And Disinfection

ABSTRACT Objective: Home nebulizers are routinely used in the treatment of patients with cystic fibrosis (CF). This study aims to evaluate the contamination of nebulizers used for CF patients, that are chronically colonized by Pseudomonas aeruginosa, and the association of nebulizer contamination with cleaning, decontamination and drying practices. Methods: A cross-sectional, observational, multicenter study was conducted in seven CF reference centers in Brazil to obtain data from medical records, structured interviews with patients/caregivers were performed, and nebulizer’s parts (interface and cup) were collected for microbiological culture. Results: overall, 77 CF patients were included. The frequency of nebulizer contamination was 71.6%. Candida spp. (52.9%), Stenotrophomonas maltophilia (11.9%), non-mucoid P. aeruginosa (4.8%), Staphylococcus aureus (4.8%) and Burkholderia cepacia complex (2.4%) were the most common isolated pathogens. The frequency of nebulizers’ hygiene was 97.4%, and 70.3% of patients reported cleaning, disinfection and drying the nebulizers. The use of tap water in cleaning method and outdoor drying of the parts significantly increased (9.10 times) the chance of nebulizers’ contamination. Conclusion: Despite the high frequency hygiene of the nebulizers reported, the cleaning and disinfection methods used were often inadequate. A significant proportion of nebulizers was contaminated with potentially pathogenic microorganisms for CF patients. These findings support the need to include patients/caregivers in educational programs and / or new strategies for delivering inhaled antibiotics.

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Inhaled hypertonic saline produces small increases in lung function in patients with cystic fibrosis

The Journal of Pediatrics ◽

10.1016/j.jpeds.2006.04.030 ◽

2006 ◽

Vol 149 (1) ◽

pp. 142 ◽

Cited By ~ 3

Author(s):

Felix Ratjen

Keyword(s):

Cystic Fibrosis ◽

Lung Function ◽

Hypertonic Saline

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Public Beliefs About Obesity Relative to Other Major Health Risks: Representative Cross-Sectional Surveys in the USA, the UK, and Germany

Annals of Behavioral Medicine ◽

10.1093/abm/kax003 ◽

2018 ◽

Vol 52 (4) ◽

pp. 273-286 ◽

Cited By ~ 11

Author(s):

Jutta Mata ◽

Ralph Hertwig

Keyword(s):

Health Risks ◽

Cross Sectional ◽

Major Health ◽

Public Beliefs ◽

The Usa ◽

The Uk

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International Comparison of the Levels and Potential Correlates of Objectively Measured Sedentary Time and Physical Activity among Three-to-Four-Year-Old Children

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph16111929 ◽

2019 ◽

Vol 16 (11) ◽

pp. 1929 ◽

Cited By ~ 5

Author(s):

Kaiseree Dias ◽

James White ◽

Russell Jago ◽

Greet Cardon ◽

Rachel Davey ◽

...

Keyword(s):

Physical Activity ◽

Parental Education ◽

Sedentary Time ◽

Linear Regression Models ◽

Cross Sectional ◽

The Usa ◽

Day Of The Week ◽

The Uk ◽

Objectively Measured ◽

Multilevel Linear Regression

Physical activity (PA) patterns track from childhood through to adulthood. The study aimed to determine the levels and correlates of sedentary time (ST), total PA (TPA), and moderate-to-vigorous PA (MVPA) in preschool-aged children. We conducted cross-sectional analyses of 1052 children aged three-to-four-years-old from six studies included in the International Children’s Accelerometry Database. Multilevel linear regression models adjusting for age, gender, season, minutes of wear time, and study clustering effects were used to estimate associations between age, gender, country, season, ethnicity, parental education, day of the week, time of sunrise, time of sunset, and hours of daylight and the daily minutes spent in ST, TPA, and MVPA. Across the UK, Switzerland, Belgium, and the USA, children in our analysis sample spent 490 min in ST per day and 30.0% and 21.2% of children did not engage in recommended daily TPA (≥180 min) and MVPA (≥60 min) guidelines. There was evidence for an association between all 10 potential correlates analyzed and at least one of the outcome variables; average daily minutes spent in ST, TPA and/or MVPA. These correlates can inform the design of public health interventions internationally to decrease ST and increase PA in preschoolers.

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