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Pulmonary function deficits in newborn screened infants with cystic fibrosis managed with standard UK care are mild and transient
With the advent of novel designer molecules for cystic fibrosis (CF) treatment, there is huge need for early-life clinical trial outcomes, such as infant lung function (ILF). We investigated the degree and tracking of ILF abnormality during the first 2â
years of life in CF newborn screened infants.
Forced expiratory volume in 0.5â
s (FEVâ.â
), lung clearance index (LCI) and plethysmographic functional residual capacity were measured at âŒ3â
months, 1â
year and 2â
years in 62 infants with CF and 34 controls.
By 2â
years there was no significant difference in FEVâ.â
z-score between CF and controls, whereas mean LCI z-score was 0.81 (95% CI 0.45â1.17) higher in CF. However, there was no significant association between LCI z-score at 2â
years with either 3-month or 1-year results. Despite minimal average group changes in any ILF outcome during the second year of life, marked within-subject changes occurred. No child had abnormal LCI or FEVâ.â
on all test occasions, precluding the ability to identify âhigh-riskâ infants in early life.
In conclusion, changes in lung function are mild and transient during the first 2â
years of life in newborn screened infants with CF when managed according to a standardised UK treatment protocol. Their potential role in tracking disease to later childhood will be ascertained by ongoing follow-up
European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre
A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those who are not. Key to the effectiveness of the specialist CF Centre is the multidisciplinary team (MDT), which should include consultants, clinical nurse specialist, microbiologist, physiotherapist, dietitian, pharmacist, clinical psychologist, social worker, clinical geneticist and allied healthcare professionals, all of whom should be experienced in CF care. Members of the MDT are also expected to keep up to date with developments in CF through continued professional development, attendance at conferences, auditing and involvement in research. Specialists CF Centres should also network with other Centres both nationally and internationally, and feed Centre data to registries in order to further the understanding of the disease. This paper provides a framework for the specialist CF Centre, including the organisation of the Centre and the individual roles of MDT members, as well as highlighting the value of CF organisations and disease registries
Importinâ7 mediates glucocorticoid receptor nuclear import and is impaired by oxidative stress, leading to glucocorticoid insensitivity
This article was supported by the NIHR Respiratory Disease Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London
Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis
Lung inflammation and injury is critical in cystic fibrosis. An ideal antiinflammatory agent has not been identified but inhaled corticosteroids are widely used despite lack of evidence