Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues

This is the third in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family. CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey

Grossesses géméllaires chez les femmes atteintes de mucoviscidose

LYON1-BU Santé (693882101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Etude de l'atteinte myocardique au cours du lupus érythémateux systémique (à propos de 34 patients)

LYON1-BU Santé (693882101) / SudocSudocFranceF

LE TEST DE LA SUEUR CHEZ L'ADULTE (REVUE DE LA LITTERATURE)

LYON1-BU Santé (693882101) / SudocPARIS-BIUM (751062103) / SudocPARIS-Bib. Serv.Santé Armées (751055204) / SudocSudocFranceF

Aspects actuels et tableaux hématologiques des carences en vitamine B12 (à propos de 31 cas)

LYON1-BU Santé (693882101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Stress oxydatif et mucoviscidose (profil clinique et biologique de 312 patients)

LYON1-BU Santé (693882101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Recueil des caractéristiques des patients atteints d'accidents vasculaires cérébraux (A.V.C.) admis au service d'accueil d'urgence (S.A.U.) du Centre hospitalier régional d'Orléans, afin d'évaluer leur mode de prise en charge initiale et intra-hospitalier en vue de définir les besoins nécessaires à la création d'une filière neuro-vasculaire

TOURS-BU Médecine (372612103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation

International audienceLow bone mineral density (BMD) is a common problem in adults with cystic fibrosis (CF), the etiology of which is multifactorial. In this study, we provide the first evidence that ivacaftor improves BMD in CF patients carrying the p.Gly551Asp mutation. Consistently, in vitro experiments with TNF-α-stimulated primary F508del-CFTR osteoblasts demonstrated that correction of p.Phe508del-CFTR markedly decreased RANKL protein production, a major factor of bone resorption. These clinical and fundamental observations suggest that rescue of mutated CFTR protein improves bone remodeling and support the link between CFTR and bone cell physiology. These findings represent a step forward in the development of potential new therapies for CF-related bone disease

Paternity in men with cystic fibrosis: a retrospective survey in France

Background Because more patients reach adulthood, new questions as “what about having a child and/or paternity responsibility?” arose. Method We performed a retrospective investigation based on the French CF registry. The context of the paternity and the health status of fathers were recorded. A comparison with clinical status of non-father patients and a compilation of follow-up data to evaluate its impact were done. Results Forty-eight men had 69 children. One fourth was said to be natural conceptions, 69% needed assisted reproduction techniques. No child had CF. Clinical status of men was satisfactory: mean BMI was 20.9 kg/m2 and mean FEV1 and FVC were 50.5% and 69.2% of predicted, respectively. When matched to CF non-fathers, few significant differences appeared. More non-fathers were F508del/F508del (p=0.03). Fathers' sputum cultures were positive for non-Pseudomonas aeruginosa strain (p=0.05), including Staphylococcus aureus (p=0.01). Mean age at diagnosis was higher, and based on minor evidence of sterility as first symptom leading to the diagnosis of CF (p=0.01) or aspergillosis (p=0.03). The 3-year follow-up showed no degradation of the clinical status. Conclusion Men having paternity responsibility over children did not differ from the CF male population and neither did it seem to have an impact on the disease course