Journal of Cystic Fibrosis
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The Journal of Cystic Fibrosis is the official journal of the European Cystic Fibrosis Society. The journal is devoted to promoting the research and treatment of cystic fibrosis. To this end the journal publishes original scientific articles, editorials, case reports, short communications and other information relevant to cystic fibrosis.
Journal of Cystic Fibrosis
1w ago
Nasal nitric oxide (nNO) is known to be reduced in people with primary ciliary dyskinesia (PwPCD) and the measurement is used in PCD's diagnostic workup [1]. It was found, that nNO is not only reduced in PCD, but also in people with cystic fibrosis (PwCF), and in chronic rhinosinusitis (CRS) even without underlying CF and nasal polyps [2,3]. It is increased in people with allergic rhinitis and asthma compared to healthy controls (HC) [2]. People with reduced nNO levels and a history of lung disease should therefore not only be evaluated for PCD, but also for CF and other diseases associated wi ..read more
Journal of Cystic Fibrosis
1w ago
Cystic fibrosis (CF), the most frequent autosomal recessive disease in the Caucasian population, is caused by mutations that disrupt chloride (Cl−) and bicarbonate transport by CFTR protein, a plasma membrane channel mainly expressed in epithelial cells of respiratory and gastrointestinal systems [1,2]. Most people with CF (pwCF) are affected by chronic obstructive pulmonary disease that is the result of the vicious cycle of respiratory bacterial infection, mucus hypersecretion, and inflammation ..read more
Journal of Cystic Fibrosis
1w ago
The European Cystic Fibrosis Society (ECFS) and its Standards of Care Committee and Diagnostic Network Working Group have launched a project to update recommendations on the diagnosis and management of cystic fibrosis transmembrance conductance regulator (CFTR)-related disorders (CFTR-RD). Two of the four planned documents addressing the general considerations of the definition and management of CFTR-RD have been published in the Journal of Cystic Fibrosis [1,2]. This third paper of the series examines in more depth the characteristics of the well established disorders that are either occasion ..read more
Journal of Cystic Fibrosis
1w ago
Cystic Fibrosis Transmembrane conductance Regulator (CFTR) modulators are the first drugs to directly target the underlying defect of the disease, and have been transforming treatment of people with Cystic Fibrosis (pwCF). The combination of elexacaftor-tezacaftor-ivacaftor (ETI) is the newest and most effective CFTR modulating medicine [1]. ETI is administered at a fixed dose in adolescents and adults (aged 12 years and older) and adjusted to age and/or weight in the pediatric population (under 12 years) [1 ..read more
Journal of Cystic Fibrosis
1w ago
Extrapulmonary manifestations are a major source of morbidity and poor quality of life for people with cystic fibrosis (pwCF), and include pancreatic exocrine and endocrine dysfunction, intestinal obstruction, chronic liver disease, reproductive dysfunction, and significant nutritional deficiencies [1,2]. Small, single center studies have reported that iron deficiency (ID) is a common extrapulmonary comorbidity in pwCF, with a reported prevalence of 40–80 % [3–5]. The presence of ID increases with advancing age and has been associated with markers for poor outcomes, including shorter time to p ..read more
Journal of Cystic Fibrosis
1w ago
Over the past thirty years, there have been remarkable incremental improvements in cystic fibrosis (CF) care, resulting in improved patient outcomes and a notable increase in median life expectancy from 28 years in 1990 to 45 years in 2018 [1,2]. However, the management of CF poses a significant burden, with adults typically dedicating almost 2 h daily to complete their CF treatments [3 ..read more
Journal of Cystic Fibrosis
1w ago
Based on advances in therapy and care management, people with cystic fibrosis (CF) are living longer and healthier lives [1].. The median predicted survival in the United States (U.S.) is now in the mid-50 s, and most PwCF in the U.S. are adults. Thus, more PwCF have both general and CF-specific sexual and reproductive health (SRH) questions and are contemplating their reproductive futures [2,3 ..read more
Creation of a CF-specific antibiotic spectrum index (ASI) as an antimicrobial stewardship initiative
Journal of Cystic Fibrosis
2w ago
Antibiotics are routinely included in the treatment of cystic fibrosis (CF)-related pulmonary exacerbations (PEx) despite limited data to guide optimal selection. Unsurprisingly, this dearth of evidence has led to substantial variability in antibiotic choice[1,2]. Antibiotics are frequently selected based on prior successful PEx treatment regimens or on prior respiratory culture and susceptibility results. Although antibiotic use among people with CF (PwCF) has been associated with improved clinical outcomes[3,4], there are growing concerns related to antimicrobial resistance and other importa ..read more
Journal of Cystic Fibrosis
2w ago
People living with cystic fibrosis (CF) often report a high symptom burden comprising physical (e.g., cough, dyspnea), psychosocial (e.g., depression, anxiety) and existential (e.g., fear of dying) symptoms [1–6]. Advances in CF care such as highly effective modulator therapy (HEMT), improved airway clearance therapies, nutritional support, and lung transplantation are projected to add decades to the lives of people with CF, with a median predicted survival of 56 years [7,8]. HEMT refers to a specific combination of drugs effective in correcting the malfunctioning protein made by the CFTR gene ..read more