Diagnosis & Disease Information

Human microbiota

New Study Shows Altered Gut-Lung Axis in Children With Cystic Fibrosis

A recent study published in Gut Microbes identified correlations between dietary habits, gastrointestinal and respiratory microbiota, lung function, and pulmonary exacerbations in children with cystic fibrosis (CF). For the purpose of this prospective, cross-sectional, controlled observational study, the researchers recruited 82 participants at an Australian tertiary pediatric hospital. They compared a cohort of children with…

High throughput sequencing

HTS Technology May Identify Patients for CF Disease-Modifying Therapies

A study conducted by researchers from Brazil showed that high-throughput sequencing technology (HTS) could be useful to establish a definitive molecular diagnosis of cystic fibrosis (CF). “HTS technology applied to the identification of pathogenic variants associated with the development of this disease allows for the introduction of therapy with CFTR modulators that favour better patient…

Xenleta bottles

Positive Topline Results Announced for Lefamulin in Adults With CF

Nabriva Therapeutics has announced positive topline results from a phase 1 clinical trial assessing the safety and pharmacokinetics of lefamulin (Xenleta®) in adult patients with cystic fibrosis (CF). In a press release, Christine Guico-Pabia, MD, MBA, MPH, chief medical officer of Nabriva Therapeutics, was thrilled to share the new data. “The data indicate that the…

Doctor listening to patient lungs

Positive Results Seen With ETI Treatment in Patients With CF

The treatment of cystic fibrosis (CF) with elexacaftor/tezacaftor/ivacaftor (ETI) might be associated with increased lung function and body mass index (BMI), fewer disease exacerbations, and only mild side effects, according to a study published in the journal Pharmacological Research & Perspectives. This multicenter longitudinal observational study gathered 20 Dutch patients with CF who received ETI…

palliative

Specialist Palliative Care Enhances Quality of Life for Adults With CF

The addition of specialist palliative care to standard clinic visits for adults with cystic fibrosis (CF) is feasible, acceptable, and efficacious, according to the results of a pilot, single-site, equal-allocation, randomized controlled study published in the Journal of Palliative Medicine. The investigators sought to evaluate the feasibility, acceptability, and perceived efficacy of longitudinal specialist palliative…

Cystic Fibrosis

Challenged Bicarbonate Excretion May Be a Biomarker of CFTR Function in CF

A new study has found an association between challenged urine bicarbonate excretion and clinical characteristics in cystic fibrosis (CF). The study, published in the Annals of Internal Medicine, observed partially restored renal cystic fibrosis transmembrane conductance regulator (CFTR) function in patients with CF undergoing therapy with elexacaftor/tezacaftor/ivacaftor. “The objective of this study was to conduct…

Happy lung patient

Novel Cystic Fibrosis Drug Preserved Lung Function in a 6-Week Study

Individuals with cystic fibrosis (CF) aged 12 years and older on the CF transmembrane conductance regulator (CFTR) modulator drug Trikafta® did not experience a decline in their lung function when they ceased taking hypertonic saline or dornase alfa, according to a news release.  The properties of Trikafta became apparent after a 6-week study funded by…

Bone mineral density

Positive Results Seen With Trikafta Treatment in Cystic Fibrosis

A pilot study evaluating the benefits of Trikafta treatment for cystic fibrosis (CF) indicates a significant improvement in bone mineral density (BMD), body composition, and exercise capacity, according to a new article published in the journal Pediatric Pulmonology.  The study design included 9 CF patients treated with Trikafta and 9 control CF patients who never…

Decreasing arrows

CF Incidence May Be Lower Than Previously Reported and Varies Widely

A new study has employed contemporary diagnostic algorithms for cystic fibrosis (CF) in North America and found that incidence rates may be lower than previously reported. The study, published in the Journal of Cystic Fibrosis, also found that the incidence rates varied widely within North America, which could impact future resource planning and usage. “The…

Asian American woman with stomach pain

Patients With CF Experience Significant GI Manifestations at All Ages

Gastrointestinal symptoms are present in patients with cystic fibrosis (CF) across all ages, indicating that patients generally suffer from a high gastrointestinal disease burden, according to a study published in the Journal of Cystic Fibrosis.  CF is characterized by thickened secretions and impaired ciliary function in the lungs, the luminal gut, and the pancreas. Although…

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