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Cystic Fibrosis

[Parenthood challenged by cystic fibrosis. Experience of affected parents].

May 23, 2022

Medical progress affords patients with cystic fibrosis (CF) the opportunity to become parents.To assess the psychological issues of parenthood in persons with cystic fibrosis.Semi-structured interview...

Host and pathogen response to bacteriophage engineered against Mycobacterium abscessus lung infection.

May 16, 2022

Two mycobacteriophages were administered intravenously to a male with treatment-refractory Mycobacterium abscessus pulmonary infection and severe cystic fibrosis lung disease. The phages were engineer...

Pharmacological Effects of Panduratin A on Renal Cyst Development in In Vitro and In Vivo Models of Polycystic Kidney Disease.

Apr 25, 2022

Renal cyst expansion in polycystic kidney disease (PKD) involves abnormalities in both cyst-lining-cell proliferation and fluid accumulation. Suppression of these processes may retard the progression ...

Influence of Bronchoscopic Interventions on Graft Function of Double Lung Transplant Recipients due to Cystic Fibrosis.

Apr 25, 2022

Healing of bronchial anastomoses may sometimes be complicated and require bronchoscopic intervention (BI). The main aim of the study was to assess whether patients who require BI present comparable lu...

Repeatability of lung clearance index in infants with cystic fibrosis and recurrent wheeze.

Apr 18, 2022

To describe the short- and medium-term repeatability of lung clearance index (LCI ) in infants and calculate the number of patients needed to enrol in a study (N) using LCI as a primary outcome.An 8-m...

Genetic evidence supports the development of SLC26A9 targeting therapies for the treatment of lung disease.

Apr 11, 2022

Over 400 variants in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) are CF-causing. CFTR modulators target variants to improve lung function, but marked variability in response ex...

Pathophysiology of Lung Disease and Wound Repair in Cystic Fibrosis.

Apr 04, 2022

Cystic fibrosis (CF) is an autosomal recessive, life-threatening condition affecting many organs and tissues, the lung disease being the chief cause of morbidity and mortality. Mutations affecting the...

The NSAID glafenine rescues class 2 CFTR mutants via cyclooxygenase 2 inhibition of the arachidonic acid pathway.

Mar 21, 2022

Most cases of cystic fibrosis (CF) are caused by class 2 mutations in the cystic fibrosis transmembrane regulator (CFTR). These proteins preserve some channel function but are retained in the endoplas...

A prospective randomised controlled mixed-methods pilot study of home monitoring in adults with cystic fibrosis.

Mar 14, 2022

Home monitoring (HM) is able to detect more pulmonary exacerbations (PEx) than routine care (RC) in individuals with cystic fibrosis (CF), but there is currently no evidence for benefits in health out...

Nutrition in Cystic Fibrosis-Some Notes on the Fat Recommendations.

Feb 28, 2022

Nutrition is important in cystic fibrosis (CF) because the disease is associated with a higher energy consumption, special nutritional deficiencies, and malabsorption mainly related to pancreatic insu...

Xylitol as a Hydrophilization Moiety for a Biocatalytically Synthesized Ibuprofen Prodrug.

Feb 28, 2022

Biocatalyzed synthesis can be exploited to produce high-value products, such as prodrugs. The replacement of chemical approaches with biocatalytic processes is advantageous in terms of environmental p...

Do clinimetric properties of LCI change after correction of signal processing?

Feb 21, 2022

The recently described sensor-crosstalk error in the multiple-breath washout (MBW) device Exhalyzer D (Eco Medics AG, Duernten, Switzerland) could highly influence clinimetric properties and the curre...

Synthesis and bioactivity of readily hydrolysable novel cationic lipids for potential lung delivery application of mRNAs.

Feb 07, 2022

Lipid nanoparticles (LNPs) mediated mRNA delivery has gained prominence due to the success of mRNA vaccines against Covid-19, without which it would not have been possible. However, there is little cl...

Carriage and transmission of macrolide resistance genes in patients with chronic respiratory conditions and their close contacts.

Feb 07, 2022

Long-term macrolide therapy has been shown to provide benefit to those with a range of chronic respiratory conditions. However, there remain concerns about the impact of macrolide exposure on the carr...

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...