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Respiratory Medicine Congenital Lung Disease

Congenital Lung Disease

28-03-2024 Cystic Fibrosis News

Bronchial dilatation reversal in CF possible with treatment but in only a few

Treatment with elexacaftor–tezacaftor–ivacaftor reverses cylindrical bronchial dilatations in just 5% of adults with cystic fibrosis.

Editor's Choice

Primary ciliary dyskinesia: integrating genetics into clinical practice

Open Access Congenital Lung Disease Review

The growing genomic knowledge about primary ciliary dyskinesia will aid diagnosis, understanding of prognosis, and the establishment of future therapeutic trials.

Evidence-based diagnosis of bronchiectasis

Bronchiectasis is a syndrome with characteristic clinical and radiologic features. A correct diagnosis of disease and its etiology is the first step in planning an appropriate therapeutic strategy.

The lung microbiome in chronic respiratory disorders

A discussion of lung microbiome dysbiosis in various chronic respiratory disorders and the mechanisms by which these bacteria can cause or contribute to the severity of disease.

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Current Reviews

Nutrition in Pediatric Liver Disease

In liver disease, there is derangement of appetite, digestion, absorption, assimilation, storage and metabolism of both macro and micronutrients. These derangements have an impact on mortality and morbidity associated with liver diseases. In …

Fungal Respiratory Colonization and Infections in Cystic Fibrosis Patients in the Middle East

Cystic fibrosis (CF) is a fatal autosomal recessive genetic disease that systemically affects multiple organs and affects patients from different aspects of life throughout their lives [ 1 ]. Although the disease is considered a multi-organ …

Neurogastroenterology and Motility Disorders of the Gastrointestinal Tract in Cystic Fibrosis

Gastrointestinal (GI) symptoms are highly prevalent in people with Cystic Fibrosis (PwCF),correlating with worse quality of life (QOL), but objective diagnoses and targeted treatments are scarce [ 1 ]. Cystic fibrosis (CF) is a multiorgan autosomal …

Treatable traits and challenges in the clinical management of non-tuberculous mycobacteria lung disease in people with cystic fibrosis

Cystic fibrosis (CF) is a lethal inherited disease characterized by multiorgan manifestations with morbidity and mortality primarily arising from CF-related lung involvement [ 1 – 3 ]. Systemic manifestations result from the impairment of the …

Further Reading

Comparison of refugee patients with cystic fibrosis and their counterpart children from Turkey during the war

Open Access Cystic Fibrosis RESEARCH

Since the outbreak of the Syrian civil war in 2011, the population of Arab refugees in Turkey has rapidly increased. While cystic fibrosis (CF) is believed to be rare among Arabs, recent studies suggest it is underdiagnosed. This study aims to …

Elevated Eosinophil Counts in Acute Exacerbations of Bronchiectasis: Unveiling a Distinct Clinical Phenotype

Open Access Bronchiectasis BRONCHIECTASIS

Non-cystic fibrosis (Non-CF) bronchiectasis is a chronic respiratory disease characterized by bronchial dilation, which can lead to chronic cough, sputum production, and recurrent respiratory infections [ 1 ]. Previous studies have identified the …

A conceptual framework to develop a patient-reported experience questionnaire on the cystic fibrosis journey in France: the ExPaParM collaborative study

Open Access Cystic Fibrosis Research

Cystic fibrosis (CF) is a rare genetic disease for which systematic newborn screening has been implemented in France for the past 20 years. https://www.ameli.fr/assure/sante/themes/mucoviscidose/diagnostic-evolution The CF care pathway is organised …

BEAT CF pulmonary exacerbations core protocol for evaluating the management of pulmonary exacerbations in people with cystic fibrosis

Open Access Cystic Fibrosis Study protocol

Note: the numbers in curly brackets in this protocol refer to SPIRIT checklist item numbers. The order of the items has been modified to group similar items (see …