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Cystic fibrosis-related diabetes (CFRD) is the most frequent extrapulmonary complication of cystic fibrosis (CF).
The risk of vitamin E deficiency is of primary concern in cystic fibrosis patients. However, early diagnosis and routine vitamin E supplementation can lead to its normal or even high levels. In the present study, we assessed vitamin E status in a large group of cystic fibrosis patients. Moreover, we also aimed to establish determinants of its body resources in cystic fibrosis patients.
In healthy lungs, epithelial sodium channel (ENaC) is regulated by short, palate, lung, and nasal clone 1 (SPLUNC1). In cystic fibrosis (CF), ENaC is hyperactivated in part due to a loss of SPLUNC1 function. We have developed SPX-101 to replace the lost function of SPLUNC1 in the CF lung.
The aim of this study was to describe the newest development in cystic fibrosis (CF) care, CF transmembrane conductance regulator (CFTR) modulator therapies.
Cystic fibrosis diagnosis is dependent on the chloride ion concentration in the sweat test (≥60mEq/mL - recognized as the gold standard indicator for cystic fibrosis diagnosis). Moreover, the salivary glands express the CFTR protein in the same manner as sweat glands. Given this context, the objective was to verify the correlation of saliva chloride concentration (SaCl) and sweat chloride concentration (SwCl), and between saliva sodium concentration (SaNa) and sweat sodium concentration (SwNa), in patient...
Cystic fibrosis is the most common genetic disorder in Caucasians. The combination of the cystic fibrosis transmembrane conductance regulator (CFTR) corrector lumacaftor / potentiator ivacaftor (LUM/IVA) has been shown to increase forced expiratory volume in 1 second (FEV1) moderately, but predominantly reduce acute exacerbation rate (AER) in Phe508del homozygous cystic fibrosis patients; however, patients with FEV1
Pulmonary hypertension (PH) is frequently found in advanced parenchymal lung diseases like cystic fibrosis (CF), but the role played by PH in the clinical outcome of CF patients remains unclear. The aim of this study is to determine the influence of PH on survival in the CF population by meta-analysis.
Individuals diagnosed with cystic fibrosis (CF) as adults represent a growing sub-population of CF cases but there are limited studies describing their characteristics and prognosis.
The chronic airway infections with Pseudomonas aeruginosa determine morbidity in most individuals with cystic fibrosis (CF). P. aeruginosa may persist for decades in CF lungs which provides the rare opportunity to study the long-term within-host evolution of a bacterial airway pathogen.
Cystic fibrosis (CF) is an inherited, chronic, progressive condition affecting around 10,000 individuals in the UK and over 70,000 worldwide. Survival in CF has improved considerably over recent decades and it is important to provide up-to-date information on patient prognosis.
The Australian Cystic Fibrosis Data Registry is positioning itself as an exemplar of a rare disease registry for the future. While it continues to inform cystic fibrosis (CF) clinicians of patterns of CF disease and quality of care, its capability is increasing as a resource for further research into CF subpopulations, as a platform for clinical trials, and as an interface for patient experiences.
Cystic fibrosis is an inherited recessive disorder of chloride transport that is characterised by recurrent and persistent pulmonary infections from resistant organisms that result in lung function deterioration and early mortality in sufferers.Meticillin-resistant Staphylococcus aureus (MRSA) has emerged as, not only an important infection in people who are hospitalised, but also as a potentially harmful pathogen in cystic fibrosis. Chronic pulmonary infection with MRSA is thought to confer people with cys...
Pseudomonas aeruginosa is the predominant pathogen responsible of chronic colonization of the airways in Cystic Fibrosis patients. There are few European data about antibiotic susceptibility evolution of Pseudomonas aeruginosa in Cystic Fibrosis patients.
Analysis of cell-free fetal DNA in maternal plasma is very promising for early diagnosis of monogenic diseases. However, it has been limited by the need to set up patient- or disease-specific custom-made approaches. Here we propose a universal test based on fluorescent multiplex PCR and size fragment analysis for an indirect diagnosis of cystic fibrosis (CF).
Patients with cystic fibrosis are characterized by an increased risk of nutrient malabsorption and inflammation, which may influence body composition. We examined the differences in body composition between patients with cystic fibrosis and healthy controls and how body composition differences may impact disease risk and mortality.
Infection with Pseudomonas aeruginosa (Pa) with a chronic phenotype is associated with antibiotic eradication therapy (AET) failure. Our objective was to determine whether higher levels of Pa (detected using qPCR) prior to culture positivity were associated with AET failure in pediatric CF patients.
Cystic fibrosis and its employment corollaries have received little attention despite the fact that complications of CF represent numerous theorized barriers to optimal career outcomes.
Cystic fibrosis is the most common life-limiting autosomal recessive genetic disorder in white populations. Distal intestinal obstruction syndrome (DIOS) is an important morbidity in cystic fibrosis. It is the result of the accumulation of viscid faecal material within the bowel which combines with thick, sticky mucus produced in the intestines of people with cystic fibrosis. The intestine may be completely blocked (complete DIOS) or only partially blocked (incomplete DIOS). Once a diagnosis of DIOS has bee...
Cystic fibrosis is the most common lethal genetic disease in the Caucasian population, characterized by CFTR gene mutations, which is a chloride channel. Whereas this gene has been known since 1989, the curative therapeutic solutions proposed to patients remain limited. New therapeutic strategies are therefore being explored, such as those targeting miRNA participating in the regulation of target mRNA expression. This review focuses on the involvement of miRNA in cystic fibrosis including ion channel contro...
Cystic Fibrosis (CF) patients of Hispanic origin are the largest growing minority, representing 8.5% of CF patients in the United States. No national survival analysis of this group has ever been undertaken.
Nasal irrigations with antibiotics are used to eradicate Pseudomonas aeruginosa from the upper airways in patients with cystic fibrosis (CF) and thereby avoid lung colonisations; nevertheless, the efficacy is uncertain.
Diabetes is associated with worse cystic fibrosis (CF) outcomes. The CFTR potentiator ivacaftor is suggested to improve glucose homeostasis in individuals with CF.