Obstructive Lung Disease

Biomarkers of Pancreatic Function in CF Improved With Lumacaftor/Ivacaftor

Biomarkers of Pancreatic Function in CF Improved With Lumacaftor/Ivacaftor

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Improvements in pancreatic function biomarkers, including increases in fecal elastase-1 and decreases in serum immunoreactive trypsinogen, were seen in patients treated with lumacaftor and ivacaftor.

Buying Time to First Exacerbation in Non-Cystic Fibrosis Bronchiectasis

Buying Time to First Exacerbation in Non-Cystic Fibrosis Bronchiectasis

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Inhaled liposomal ciprofloxacin is associated with a longer median time to first pulmonary exacerbation vs placebo in patients with non-cystic fibrosis bronchiectasis and chronic P aeruginosa lung infection requiring antibiotic therapy.

Cystic Fibrosis May Be a Risk Factor for Hemiplegic Migraine and Stroke

Cystic Fibrosis May Be a Risk Factor for Hemiplegic Migraine and Stroke

Due to brain excitability and hypoxia from coughing, patients with cystic fibrosis have a propensity for cortical spreading depression and hemiplegic migraine.

Primary Ciliary Dyskinesia: Practical Pearls for Diagnosis

Primary Ciliary Dyskinesia: Practical Pearls for Diagnosis

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Recent guidelines set forth by an interdisciplinary panel assembled by the American Thoracic Society sought to demystify primary ciliary dyskinesia and address 4 main diagnostic questions.

Restrictive, Obstructive Lung Disease Linked to Dementia Risk

Restrictive, Obstructive Lung Disease Linked to Dementia Risk

Associations were seen for both Alzheimer's disease-related dementia and cerebrovascular etiologies.

Bronchiectasis Severity Associated With Pulmonary Vascular Pruning

Bronchiectasis Severity Associated With Pulmonary Vascular Pruning

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Mild bronchiectasis in smokers is associated with distal pulmonary vascular pruning.

Hypertonic Saline Inhalation Safety Tested in Infants With Cystic Fibrosis

Hypertonic Saline Inhalation Safety Tested in Infants With Cystic Fibrosis

In infants with cystic fibrosis, preventive inhalation with hypertonic saline during the first months of life is safe and well tolerated and results in clinical improvements, according to a new study.

Multiple Breath Washout Test Inaccuracies Revealed Via Over-Reader Assessment

Multiple Breath Washout Test Inaccuracies Revealed Via Over-Reader Assessment

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Including invalid data decreases the validity of multiple breath washout measurements in clinical trials involving adults with bronchiectasis.

VX-445-Tezacaftor-Ivacaftor May Improve CFTR in Cystic Fibrosis

VX-445-Tezacaftor-Ivacaftor May Improve CFTR in Cystic Fibrosis

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VX-445-tezacaftor-ivacaftor led to noticeable improvements in Phe508del CFTR protein function.

Cystic Fibrosis Lung Function Improved With Triple Therapy

Cystic Fibrosis Lung Function Improved With Triple Therapy

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Adding VX-659 to tezacaftor and ivacaftor therapy improved lung function in patients with cystic fibrosis.

CFTR Dysfunction Likely Associated With Worsened Airway Disease in Smokers

CFTR Dysfunction Likely Associated With Worsened Airway Disease in Smokers

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Cystic fibrosis transmembrane conductance regulator dysfunction is associated with worsened airway disease in patients with COPD.

Novel Inhaled Treatment for Cystic Fibrosis Granted Orphan Drug Designation

Novel Inhaled Treatment for Cystic Fibrosis Granted Orphan Drug Designation

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The FDA has granted Orphan Drug Designation to a novel inhaled treatment that could potentially improve lung function in patients with cystic fibrosis.

CF Severity Predicted by Forskolin-Induced Swelling in Intestinal Organoids

CF Severity Predicted by Forskolin-Induced Swelling in Intestinal Organoids

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Forskolin-induced swelling in intestinal organoids is a clinically relevant biomarker of disease severity in infants diagnosed with cystic fibrosis.

Does Monotherapy With Correctors Have Clinical Benefit in Cystic Fibrosis?

Does Monotherapy With Correctors Have Clinical Benefit in Cystic Fibrosis?

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Evidence is lacking that demonstrates that monotherapy with correctors has a clinically important effect on individuals with cystic fibrosis who have 2 copies of the F508del mutation.

Ivacaftor Improves Insulin Secretion in Children With Cystic Fibrosis

Ivacaftor Improves Insulin Secretion in Children With Cystic Fibrosis

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First-phase and glucose-potentiation of arginine-induced insulin secretion as assessed by acute C-peptide responses improved after the initiation of ivacaftor in children with cystic fibrosis.

Is There an "Ideal" Cystic Fibrosis Candidate for Lung Transplantation?

Is There an "Ideal" Cystic Fibrosis Candidate for Lung Transplantation?

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Lung transplantation can extend survival in patients with cystic fibrosis who have compromised lung function and increasing exacerbations.

Lung Function in Primary Ciliary Dyskinesia Affected at Early Age

Lung Function in Primary Ciliary Dyskinesia Affected at Early Age

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Lung function in patients with primary ciliary dyskinesia was reduced compared with reference values from patients with cystic fibrosis in both sexes and in all age groups.

Kalydeco Approved to Treat Cystic Fibrosis in Children 12 to <24 Months

Kalydeco Approved to Treat Cystic Fibrosis in Children 12 to <24 Months

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The approval was supported by data from ARRIVAL (N=25), an ongoing Phase 3 open-label safety study in children with CF aged 12 to <24 months with 1 of 10 CFTR gene mutations (G551D, G178R, S549N, S549R, G551S, G1244E, S1251N, S1255P, G1349D or R117H).

Predictors for Survival Rates for Adult-Diagnosed Cystic Fibrosis

Predictors for Survival Rates for Adult-Diagnosed Cystic Fibrosis

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In adult-diagnosed cystic fibrosis, diabetes, lung function, and age at diagnosis may predict patient survival.

Orkambi Approved to Treat Younger Cystic Fibrosis Patients

Orkambi Approved to Treat Younger Cystic Fibrosis Patients

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The approval was based on data from a 24-week, open-label, Phase 3 study in patients aged 2 to 5 years.

Anaerobic Bacteria in Cystic Fibrosis Airways Associated With Milder Disease

Anaerobic Bacteria in Cystic Fibrosis Airways Associated With Milder Disease

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A higher prevalence of anaerobic bacteria in cystic fibrosis was associated with higher lung function, less use of antibiotics, increased BMI, pancreatic sufficiency, and no requirement for insulin.

Smoking Marijuana May Increase Cough, Sputum Production, and Wheezing

Smoking Marijuana May Increase Cough, Sputum Production, and Wheezing

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Results of a pooled analysis of cross-sectional studies demonstrated an increase in cough, sputum production, wheezing, and dyspnea from marijuana use.

Azithromycin Reduced Pulmonary Exacerbation Risk in Cystic Fibrosis

Azithromycin Reduced Pulmonary Exacerbation Risk in Cystic Fibrosis

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Compared with placebo, the risk for a pulmonary exacerbation was reduced by 44% in participants with cystic fibrosis who received azithromycin.

<i>Aspergillus</i> Common in Bronchiectasis Pulmonary Mycobiome, Associated With Exacerbations

Aspergillus Common in Bronchiectasis Pulmonary Mycobiome, Associated With Exacerbations

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Bronchiectasis-associated genera included Aspergillus, Cryptococcus, Clavispora, Botrytis, and Alternaria.

Ivacaftor Decreases Mortality, Hospitalization in Cystic Fibrosis

Ivacaftor Decreases Mortality, Hospitalization in Cystic Fibrosis

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Ivacaftor decreased the risk for death, transplantation, hospitalization, and pulmonary exacerbation in patients with cystic fibrosis.

Lung Function in Cystic Fibrosis Affected by Sputum Viscoelasticity and Poor Clearance

Lung Function in Cystic Fibrosis Affected by Sputum Viscoelasticity and Poor Clearance

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Sputum viscoelastic properties were associated with lung function and disease status in patients with cystic fibrosis.

Novel Cystic Fibrosis Treatment Granted Fast Track Status

Novel Cystic Fibrosis Treatment Granted Fast Track Status

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Proteostasis Therapeutics is developing the combination treatment which includes a novel transmembrane conductance regulator (CFTR) amplifier (PTI-428), a third generation corrector (PTI-801) and a potentiator (PTI-808).

Cystic Fibrosis-Related Pathogen Risk With Chronic Azithromycin Use

Cystic Fibrosis-Related Pathogen Risk With Chronic Azithromycin Use

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Patients with cystic fibrosis who took azithromycin had a significantly lower risk for detection of new nontuberculous mycobacteria, methicillin-resistant Staphylococcus aureus, and Burkholderia cepacia complex.

Cystic Fibrosis Foundation Recommends CFTR Modulator Therapy in Select Patients

Cystic Fibrosis Foundation Recommends CFTR Modulator Therapy in Select Patients

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CFTR modulator therapy recommended in certain patients with cystic fibrosis.

CFTR Modulator Therapy With Tezacaftor-Ivacaftor in Cystic Fibrosis

CFTR Modulator Therapy With Tezacaftor-Ivacaftor in Cystic Fibrosis

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Effective cystic fibrosis transmembrane conductance regulator modulator therapy may be of benefit in subsets of patients with cystic fibrosis.

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