The expanded French compassionate programme for elexacaftor-tezacaftor-ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study.

Background: Elexacaftor-tezacaftor-ivacaftor has been approved in Europe for people with cystic fibrosis with at least one F508del CFTR variant. Additionally, it is approved by the US Food and Drug Administration (FDA) for people with cystic fibrosis with at least one of 177 rare variants. The aims of this study were to describe the clinical response to elexacaftor-tezacaftor-ivacaftor for people with cystic fibrosis without a F508del CFTR variant in France and to determine CFTR variant responsiveness to elexacaftor-tezacaftor-ivacaftor based on the observed clinical response.

Multisystemic Effects of Elexacaftor-Tezacaftor-Ivacaftor in Adults with Cystic Fibrosis and Advanced Lung Disease.

Limited data exist on the safety and effectiveness of elexacaftor-tezacaftor-ivacaftor (ETI) in people with cystic fibrosis (pwCF) and advanced lung disease. To evaluate the effects of ETI in an unselected population of pwCF and advanced lung disease. A prospective observational study, including all adults aged 18 years and older with percentage predicted forced expiratory volume in 1 second (ppFEV) ⩽ 40 who initiated ETI from December 2019 to June 2021 in France, was conducted.

CT imaging shows specific pancreatic abnormalities in persons with cystic fibrosis related diabetes.

Cystic fibrosis related diabetes (CFRD) is observed in 20-50% of adults with cystic fibrosis (CF). Pancreas abnormalities on imaging, e.g.

Implementing advanced practice nursing in France: A country-wide survey 2 years after its introduction.

Objectives: To examine the characteristics of the first Advanced Practice Nurses in France and to compare the French model to international standards.

Background: Common barriers and facilitators to their integration in healthcare provision have been identified internationally. In France, the legislative framework was introduced in 2016, and the first graduates entered the workforce in 2019.

The Changing Epidemiology of Cystic Fibrosis: The Implications for Adult Care.

Cystic fibrosis (CF) is a genetic disease in which mutations in the gene encoding for the CF transmembrane conductance regulator protein result in a multisystem disease dominated by digestive and respiratory manifestations. In the mid-20th century, CF caused death within the first years of life. Over the past decades, advances in disease management, which includes systematic neonatal screening, multidisciplinary symptomatic CF care, lung transplantation and, more recently, highly effective CF transmembrane conductance regulator modulators, have transformed the prognosis of people with CF markedly.

CFTR Modulators in People with Cystic Fibrosis: Real-World Evidence in France.

Cystic fibrosis (CF) is a rare genetic multisystemic disease, the manifestations of which are due to mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) protein and can lead to respiratory insufficiency and premature death. CFTR modulators, which were developed in the past decade, partially restore CFTR protein function. Their clinical efficacy has been demonstrated in phase 3 clinical trials, particularly in terms of lung function and pulmonary exacerbations, nutritional status, and quality of life in people with gating mutations (ivacaftor), homozygous for the F508del mutation (lumacaftor/ivacaftor and tezacaftor/ivacaftor), and in those with at least one F508del mutation (elexacaftor/tezacaftor/ivacaftor).

Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis.

Background: Elexacaftor-tezacaftor-ivacaftor induces rapid clinical improvement in patients with cystic fibrosis (CF) and advanced pulmonary disease, often leading to suspend the indication for lung transplantation. Yet no long-term data is available in lung transplant candidates.

Methods: Lung transplant candidates (defined as being waitlisted for lung transplantation or considered for listing within 3 months) who have initiated elexacaftor-tezacaftor-ivacaftor were identified in the French cohort of patients with CF and advanced pulmonary disease.

Use of anti-CMV immunoglobulins in lung transplant recipients: The French experience.

Rational: Pending the authorization of new anti-CMV drugs with fewer adverse effects, exploring the possibilities offered by CMV immunoglobulins (CMVIG) seems necessary. In France, access to CMVIG requires official authorization by the national Health authority and is restricted to second line rescue therapy for CMV infection/disease. The aim of this multicenter retrospective study is to describe the indications and clinical situations that justified its use in France.

Nurse coordinator roles in the management of patients with hepatocellular carcinoma: A French national survey.

Unlabelled: Hepatocellular carcinoma (HCC) mostly occurs in patients with chronic liver disease (CLD). HCC treatment may have a direct impact on CLD prognosis. HCC management can therefore become complex, involving multiple health care providers, such as oncologists, hepatologists, radiologists, and surgeons.

Influenza vaccination of pregnant women in Paris, France: Knowledge, attitudes and practices among midwives.

Introduction: In France, midwives have been authorized to prescribe vaccines since 2016. Yet vaccination coverage among pregnant women remains low. Understanding the knowledge, attitudes and practices of midwives regarding influenza vaccination could help improve coverage.

A prospective analysis of unplanned patient-initiated contacts in an adult cystic fibrosis centre.

Background: Timely response should be provided when patients contact the cystic fibrosis (CF) centre in between scheduled visits. Little data exist on unplanned patient-initiated contacts in CF adults.

Methods: A two-stage prospective study was undertaken from 1 January to 31 December 2015 at Cochin Hospital, Paris (France).

Mechanical characteristics of plastic base Ports and impact on flushing efficacy.

Background: Three types of totally implantable venous access devices, Ports, are currently in use: titanium, plastic (polyoxymethylene, POM), and mixed (titanium base with a POM shell). Physics theory suggests that the interaction between a non-coring needle (NCN, made of stainless steel) and a plastic base would lead to the stronger material (steel) altering the more malleable material (plastic).

Objectives: To investigate whether needle impacts can alter a plastic base's surface, thus potentially reducing flushing efficacy.

The role of nurses in HIV screening in health care facilities: A systematic review.

Objective: To examine nurse-driven HIV screening in various health care settings in terms of its impact on test offering, acceptance and delivery rates, nursing responsibilities, staff perceptions and long-term implementation.

Design: Systematic review.

Review Methods: The systematic review conducted in September 2014 adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement.