Cystic Fibrosis | Physician's Weekly
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Cystic Fibrosis

Assessing Treatment Options for Cystic Fibrosis Chronic Rhinosinusitis

Jan 06, 2022

“Nearly all individuals with cystic fibrosis (CF) suffer from chronic rhinosinusitis (CRS),” notes David A. Gudis, MD. “The underlying pathophysiology of CF is due to a mutation in the CF transm...

Opportunity for pharmacogenomic testing in patients with cystic fibrosis.

Jan 03, 2022

Patients with cystic fibrosis (CF) are exposed to many drugs in their lifetime and many of these drugs have Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines that are available to ...

RISK FACTORS FOR OBSTRUCTIVE SLEEP APNEA IN CYSTIC FIBROSIS.

Jan 03, 2022

Despite emerging data that suggest a high frequency and severity of obstructive sleep apnea (OSA) among patients with cystic fibrosis (CF), few of them are referred for polysomnography. Little is know...

Comparison of biosimilar Tigerase and Pulmozyme in long-term symptomatic therapy of patients with cystic fibrosis and severe pulmonary impairment (subgroup analysis of a Phase III randomized open-label clinical trial (NCT04468100)).

Dec 27, 2021

Patients with cystic fibrosis (CF) need costly medical care and adequate therapy with expensive medicinal products. Tigerase® is the first biosimilar of dornase alfa, developed by the lead Russian bi...

Perception, experience and relationship with food and eating in adults with cystic fibrosis.

Dec 20, 2021

Cystic fibrosis (CF) is a multisystem disorder that primarily affects the respiratory and gastrointestinal systems. Dietetic therapy is a prominent aspect of CF management, with patients receiving nut...

[Gene mutations in congenital bilateral absence of the vas deferens: An update].

Dec 20, 2021

Congenital bilateral absence of the vas deferens (CBAVD) is a congenital malformation of the male reproductive system and one of the important causes of obstructive azoospermia and male infertility. I...

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...