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Cystic Fibrosis

Predictive factors for premature birth and respiratory exacerbation in pregnancies of women with cystic fibrosis.

Jun 14, 2021

In this present study, the authors evaluated the predictive factors for adverse maternal-fetal outcomes in pregnancies of women with cystic fibrosis (CF). Patients were followed up by a referral cente...

Disruption of biofilms and killing of Burkholderia cenocepacia from cystic fibrosis lung using an antioxidant-antibiotic combination therapy.

Jun 14, 2021

Cystic fibrosis (CF) is a disease caused by mutations in the CF transmembrane conductance regulator. The resulting chloride and bicarbonate imbalance produces a thick static lung mucus. This mucus is ...

Self-renewal and differentiation of rat Epididymal basal cells using a novel in vitro organoid model.

Jun 10, 2021

The epididymis is composed of a pseudostratified epithelium comprised of various cell types. Studies have shown that rat basal cells share common properties with adult stem cells and begin to differen...

Lung MRI with hyperpolarised gases: current & future clinical perspectives.

Jun 10, 2021

The use of pulmonary MRI in a clinical setting has historically been limited. Whilst CT remains the gold-standard for lung imaging in many clinical indications, technical developments in ultrashort a...

Enhanced delivery of peptide-morpholino oligonucleotides with a small molecule to correct splicing defects in the lung.

Jun 10, 2021

Pulmonary diseases offer many targets for oligonucleotide therapeutics. However, effective delivery of oligonucleotides to the lung is challenging. For example, splicing mutations in the cystic fibros...

Diabetes outbreak during COVID19 lock-down in a prediabetic patient with cystic fibrosis long treated with glargine.

Jun 07, 2021

Cystic Fibrosis Related Diabetes (CFRD) is a frequent comorbidity of patients with Cystic Fibrosis (CF). A worsening of clinical conditions appears before CFRD. It has been demonstrated a decline in p...

GENERALIZED ACCELERATED RECURRENCE TIME MODEL IN THE PRESENCE OF A DEPENDENT TERMINAL EVENT.

Jun 07, 2021

Recurrent events are commonly encountered in longitudinal studies. The observation of recurrent events is often stopped by a dependent terminal event in practice. For this data scenario, we propose tw...

Change in Nutrient and Dietary Intake in European Children with Cystic Fibrosis after a 6-Month Intervention with a Self-Management mHealth Tool.

Jun 03, 2021

Cystic Fibrosis (CF) is a life-long genetic disease, causing increased energy needs and a healthy diet with a specific nutrient distribution. Nutritional status is an indicator of disease prognosis an...

CFTR Modulators: Does One Dose Fit All?

Jun 03, 2021

For many people with cystic fibrosis (pwCF), CFTR modulators will be the cornerstone of their treatment. These modulators show robust treatment effects at group level in pwCF with specific mutations. ...

Best Practices: Imaging Strategies for Reduced-Dose Chest CT in the Management of Cystic Fibrosis-Related Lung Disease.

Jun 03, 2021

Cystic fibrosis (CF) is a multisystemic life-limiting disorder. The leading cause of morbidity in CF is chronic pulmonary disease. Chest CT is the reference standard for detection of bronchiectasis. ...

Testing of aerosolized ciprofloxacin nanocarriers on cystic fibrosis airway cells infected with P. aeruginosa biofilms.

May 31, 2021

The major pathogen found in the lungs of adult cystic fibrosis (CF) patients is Pseudomonas aeruginosa, which builds antibiotic-resistant biofilms. Pulmonary delivery of antibiotics by inhalation has ...

Enabling Antibiotic Allergy Evaluations and Reintroduction of First-Line Antibiotics for Cystic Fibrosis Patients.

May 31, 2021

Cystic fibrosis (CF) patients often have a history of antibiotic adverse drug reactions (ADRs) that pose a barrier to receiving recommended first-line treatment. Targeted antibiotic allergy evaluation...

Epigenome editing of the CFTR-locus for treatment of cystic fibrosis.

May 31, 2021

Mechanisms governing the diversity of CFTR gene expression throughout the body are complex. Multiple intronic and distal regulatory elements are responsible for regulating differential CFTR expression...

Elevated sweat chloride test: is it always cystic fibrosis?

May 17, 2021

The sweat chloride test (ST) is the gold standard for cystic fibrosis (CF) diagnosis in symptomatic patients, within the newborn screening and in the follow-up of CF patients during molecular therapie...

Genetic Polymorphisms in the Host and COVID-19 Infection.

May 13, 2021

The outbreak of the COVID-19 pandemic shows a marked geographical variation in its prevalence and mortality. The question arises if the host genetic variation may (partly) affect the prevalence and mo...

SLEEP IN CHILDREN WITH CYSTIC FIBROSIS: MORE UNDER THE COVERS.

May 13, 2021

Cystic fibrosis (CF) is a chronic multisystem disease with manifestations from birth. It involves the entire respiratory system, with increased cough, and recurrent pulmonary infections, and it also l...

Association Between Non-Cystic Fibrosis Bronchiectasis and Quality of Life: A Single-Center Cross-Sectional Study.

May 10, 2021

Introduction Bronchiectasis is a chronic respiratory disease that can affect patients of all ages and significantly impact the quality of life (QOL) in patients who suffer from it. In spite of its ...

Therapeutic application of lantibiotics and other lanthipeptides: old and new findings.

May 10, 2021

Lanthipeptides are ribosomally synthesized and post translationally modified peptides, with modifications that are incorporated during biosynthesis by dedicated enzymes. Various modifications of the p...

In Vitro Antimicrobial Susceptibility Testing of Biofilm-Growing Bacteria: Current and Emerging Methods.

May 10, 2021

The antibiotic susceptibility of bacterial pathogens is typically determined based on planktonic cells, as recommended by several international guidelines. However, most of chronic infections - such a...

Evaluation of Two Chest Tomosynthesis Cystic Fibrosis Scoring Systems Using High-Resolution Computed Tomography Brody Scoring as Reference.

May 06, 2021

To evaluate two chest tomosynthesis (CTS) scoring systems for cystic fibrosis (CF), one system developed by Vult von Steyern et al. (VvS) and one system based on the Brody scoring system for high-res...