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Cystic Fibrosis

The Pseudomonas aeruginosa whole genome sequence: A 20th anniversary celebration.

Nov 29, 2021

Toward the end of August 2000, the 6.3 Mbp whole genome sequence of Pseudomonas aeruginosa strain PAO1 was published. With 5570 open reading frames (ORFs), PAO1 had the largest microbial genome sequen...

Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip: Cystic fibrosis airway chip.

Nov 22, 2021

Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), which results in impaired airway mucociliary clearanc...

CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the β-adrenergic sweat rate assay.

Nov 15, 2021

The cystic fibrosis (CF) sweat gland is defective in β-adrenergically-stimulated sweat secretion in the coil and chloride reabsorption in the duct. Whereas chloride reabsorption is regularly assessed...

Antimicrobial effects of Melaleuca alternifolia (tea tree) essential oil against biofilm-forming multi-drug resistant cystic fibrosis-associated Pseudomonas aeruginosa as a single agent and in combination with commonly nebulised antibiotics.

Oct 25, 2021

Broth microdilution assays were used to determine minimum inhibitory concentrations (MICs) and fractional inhibitory concentration indices (FICIs) of tea tree oil (TTO), tobramycin, colistin, and aztr...

The molecular evolution of function in the CFTR chloride channel.

Oct 18, 2021

The ATP-binding cassette (ABC) transporter superfamily includes many proteins of clinical relevance, with genes expressed in all domains of life. Although most members use the energy of ATP binding an...

Prevalence, variability, and predictors of sinus surgery in pediatric patients with cystic fibrosis.

Oct 18, 2021

Chronic rhinosinusitis is common among individuals with cystic fibrosis (CF) and has an impact on quality of life. Sinus surgery is a treatment option, but minimal literature exists regarding prevalen...

Impact of timing of PERT on gastrointestinal symptoms in Danish children and adolescents with CF.

Oct 11, 2021

Gastrointestinal (GI) symptoms are often reported by CF-patients. Despite a proven relation to exocrine pancreatic insufficiency (PI), it remains unclear whether GI symptoms are related to the timing ...

Olfactory dysfunction in cystic fibrosis: Impact of CFTR modulator therapy.

Oct 04, 2021

Elexacaftor-tezacaftor-ivacaftor (ETI) improves pulmonary health and chronic rhinosinusitis (CRS) for people with cystic fibrosis (PwCF), however its impact on olfaction has not been investigated. Olf...

Interventions for treating leg ulcers in people with sickle cell disease.

Sep 27, 2021

The frequency of skin ulceration makes an important contributor to the morbidity burden in people with sickle cell disease. Many treatment options are available to the healthcare professional, althoug...

Genomic analysis of Burkholderia cenocepacia isolated from a liver abscess in a patient with cystic fibrosis.

Sep 27, 2021

Burkholderia cenocepacia complex is associated with high transmissibility, virulence, and poor prognosis in cystic fibrosis (CF) patients. However, extrapulmonary infections are rare. We investigated ...

Filamentous fungi in the airway of patients with cystic fibrosis: Just spectators?

Sep 20, 2021

There are important advances in the management of bacterial infection in patients with cystic fibrosis (CF), but there are many gaps in the field of fungal infections.The aim of this study was to anal...

CFTR corrector C17 is effective in muscular dystrophy, in vivo proof of concept in LGMDR3.

Sep 13, 2021

Limb-girdle muscular dystrophy 3 (LGMDR3) is caused by mutations in the SGCA gene coding for α-sarcoglycan (SG). Together with β- γ- and δ-SG, α-SG forms a tetramer embedded in the dystrophin ass...

Ethical dilemma: ELX/TEZ/IVA or Lung Transplantation in Cystic Fibrosis and End Stage Lung Disease?

Sep 13, 2021

Cystic fibrosis is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. Novel, highly effective, modulator therapies correcting and potentiating CFTR function ar...

Phosphopantetheinyl transferase binding and inhibition by amidino-urea and hydroxypyrimidinethione compounds.

Sep 13, 2021

Owing to their role in activating enzymes essential for bacterial viability and pathogenicity, phosphopantetheinyl transferases represent novel and attractive drug targets. In this work, we examined t...

Prevalence, Characteristics and Preoperative Predictors of Chronic Pain After Double-Lung Transplantation: A Prospective Cohort Study.

Sep 06, 2021

Data on chronic pain after lung transplantation are heterogeneous. This study prospectively explored the prevalence, characteristics, consequences, and preoperative predictors of pain in lung transpla...

Survival in cystic fibrosis after acute respiratory failure supported by extracorporeal membrane oxygenation and/or invasive mechanical ventilation.

Sep 06, 2021

Despite therapeutic advances, people with cystic fibrosis (CF) develop progressive worsening and exacerbations of their lung disease, which can lead to acute respiratory failure. Historically, surviva...

Clinical Profile of Children With Cystic Fibrosis Surviving Through Adolescence and Beyond.

Sep 06, 2021

To document morbidities in adolescents with cystic fibrosis from India.Details of children with cystic fibrosis surviving beyond 15 years of age were extracted from hospital records, and analyzed.43 c...

Recombinant growth hormone therapy for cystic fibrosis in children and young adults.

Aug 26, 2021

Cystic fibrosis (CF) is an inherited condition causing disease most noticeably in the lungs, digestive tract and pancreas. People with CF often have malnutrition and growth delay. Adequate nutritional...

β-eudesmol but not atractylodin exerts an inhibitory effect on CFTR-mediated chloride transport in human intestinal epithelial cells.

Aug 26, 2021

Oriental herbal medicine with the two bioactive constituents, β-eudesmol (BE) and atractylodin (AT), has been used as a remedy for gastrointestinal disorders. There was no scientific evidence reporti...

Associations Between Glucose Tolerance, Insulin Secretion, Muscle and Fat Mass in Cystic Fibrosis.

Aug 23, 2021

A frequent comorbidity in cystic fibrosis (CF) is CF related diabetes (CFRD) caused by a gradual decline in insulin secretion. The reduction in the anabolic hormone, insulin, might explain the weight ...