Advertisement

Cystic Fibrosis

Assessing the impact of exposome on the course of chronic obstructive pulmonary disease and cystc fibrosis: The REMEDIA European Project Approach.

Aug 23, 2021

Because of the direct interaction of lungs with the environment, respiratory diseases are among the leading causes of environment-related deaths in the world. Chronic obstructive pulmonary disease (CO...

Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in in lung epithelial cells.

Aug 23, 2021

Cystic fibrosis (CF) is a monogenic disease, characterized by massive chronic lung inflammation. The observed variability in clinical phenotypes in monozygotic CF twins is likely associated with the e...

CFTR Modulator Therapy and Its Impact on Lung Transplantation in Cystic Fibrosis.

Aug 19, 2021

Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasian people and is caused by mutations in the gene encoding for the CF transmembrane conductance regulator (CFTR) protein. ...

Clotting factor concentrates for preventing bleeding and bleeding-related complications in previously treated individuals with haemophilia A or B.

Aug 19, 2021

The hallmark of severe hemophilia (A or B) is recurrent bleeding into joints and soft tissues with progressive joint damage, despite on-demand treatment. Prophylaxis has long been used, but not univer...

Review of CFTR modulators 2020.

Aug 19, 2021

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are small molecules that directly impact the CFTR protein, improving the function of the CFTR chloride and bicarbonate channel. Be...

Assessment of Lentiviral Vector Mediated CFTR Correction in Mice Using an Improved Rapid Nasal Potential Difference Measurement Protocol.

Aug 16, 2021

Cystic Fibrosis (CF) is caused by a defect in the CF transmembrane conductance regulator () gene responsible for epithelial ion transport. Nasal potential difference (PD) measurement is a well establi...

Non-invasive Diagnostic Tests in Cystic Fibrosis-Related Liver Disease: A Diagnostic Test Accuracy Network Meta-Analysis.

Aug 16, 2021

Cystic fibrosis-related liver disease (CFLD) is one of the leading causes of morbidity and mortality in cystic fibrosis (CF). Several non-invasive diagnostic methods have been proposed as screening t...

O-Specific Antigen-Dependent Surface Hydrophobicity Mediates Aggregate Assembly Type in Pseudomonas aeruginosa.

Aug 12, 2021

Bacteria live in spatially organized aggregates during chronic infections, where they adapt to the host environment, evade immune responses, and resist therapeutic interventions. Although it is known ...

Structural insights into the gating mechanism of human SLC26A9 mediated by its C-terminal sequence.

Aug 12, 2021

The human SLC26 transporter family exhibits various transport characteristics, and family member SLC26A9 performs multiple roles, including acting as Cl/HCO exchangers, Cl channels, and Na transporter...

extract elicits chloride secretion by stimulation of the intestinal TMEM16A ion channel.

Aug 09, 2021

Cucumber ( Linn. [Cucurbitaceae]) is widely known for its purgative, antidiabetic, antioxidant, and anticancer therapeutic potential. However, its effect on gastrointestinal (GI) disease is unrecognis...

Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice.

Aug 09, 2021

Cystic fibrosis (CF) is a multisystem disorder, caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. These cause a reduced secretion of chloride, a marked absorption of sodiu...

Automatic airway segmentation from computed tomography using robust and efficient 3-D convolutional neural networks.

Aug 09, 2021

This paper presents a fully automatic and end-to-end optimised airway segmentation method for thoracic computed tomography, based on the U-Net architecture. We use a simple and low-memory 3D U-Net as ...

The Cost-effectiveness of Genotyping versus Sequencing for Prenatal Cystic Fibrosis Carrier Screening.

Aug 05, 2021

We investigated the cost-effectiveness of three sequential prenatal cystic fibrosis (CF) carrier screening strategies: genotyping both partners, genotyping one partner then sequencing the second, and ...

Rapid detection by MALDI-TOF MS of isolates from cystic fibrosis patients belonging to the epidemic clones ST137 or DES.

Aug 05, 2021

spp. are increasingly reported among cystic fibrosis patients. Genotyping requires time consuming methods such as Multilocus-Sequence-Typing or Pulsed-Field-Gel-Electrophoresis. Therefore, data on t...

The Indispensable role of pharmacy services and medication therapy management in cystic fibrosis.

Aug 05, 2021

Care for people with cystic fibrosis (PWCF) is highly complex and requires a multi-disciplinary approach where the pharmacist plays a vital role. The purpose of this manuscript is to serve as a guidel...

Postnatal Ozone Exposure Disrupts Alveolar Development, Exaggerates Mucoinflammatory Responses, and Suppresses Bacterial Clearance in Developing -Tg Mice Lungs.

Aug 02, 2021

Increased levels of ambient ozone, one of the six criteria air pollutants, result in respiratory tract injury and worsening of ongoing lung diseases. However, the effect of ozone exposure on the respi...

Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis.

Aug 02, 2021

The availability of a simple, robust and non-invasive in vitro airway model would be useful to study the functionality of the cystic fibrosis transmembrane regulator (CFTR) protein and to personalize ...

Milestones of Precision Medicine: An Innovative, Multidisciplinary Overview.

Aug 02, 2021

Although the concept of precision medicine, in which healthcare is tailored to the molecular and clinical characteristics of each individual, is not new, its implementation in clinical practice has be...

The two faces of cyanide: an environmental toxin and a potential novel mammalian gasotransmitter.

Jul 29, 2021

Cyanide is traditionally viewed as a cytotoxic agent, with its primary mode of action being the inhibition of mitochondrial Complex IV (cytochrome c oxidase). However, recent studies demonstrate that ...

Genomic characterization of species isolates from chronic and occasional lung infection in cystic fibrosis patients.

Jul 26, 2021

species are increasingly being detected in cystic fibrosis (CF) patients, where they can establish chronic infections by adapting to the lower airway environment. To better understand the mechanisms ...