Objectives: Cystic fibrosis (CF) lung disease is characterised by mucus stasis, chronic infection and inflammation, causing progressive structural lung disease and eventual respiratory failure. CF airways are inhabited by an ecologically diverse polymicrobial environment with vast potential for interspecies interactions, which may be a contributing factor to disease progression. and are the most common bacterial and fungal species present in CF airways respectively and coinfection results in a worse disease phenotype.
Methods: In this review we examine existing expert knowledge of chronic co-infection with and in CF patients. We summarise the mechanisms of interaction and evaluate the clinical and inflammatory impacts of this co-infection.
Results: inhibits through multiple mechanisms: phenazine secretion, iron competition, quorum sensing and through diffusible small molecules. reciprocates inhibition through gliotoxin release and phenotypic adaptations enabling evasion of inhibition. Volatile organic compounds secreted by stimulate growth, while stimulates production of cytotoxic elastase.
Conclusion: A complex bi-directional relationship exists between and , exhibiting both mutually antagonistic and cooperative facets. Cross-sectional data indicate a worsened disease state in coinfected patients; however, robust longitudinal studies are required to derive causality and to determine whether interspecies interaction contributes to disease progression.
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http://dx.doi.org/10.1183/16000617.0011-2020 | DOI Listing |
Pediatr Pulmonol
January 2025
Beatrix Children's Hospital Department of Pediatric Pulmonology and Pediatric Allergy, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Introduction: Lumacaftor/ivacaftor (lum/iva) was introduced in the Netherlands in 2017. We investigated 1-year efficacy of lum/iva on lung function and small airway and structural lung disease evaluated by multiple breath nitrogen washout and CT scan. Additionally, we investigated effects of lum/iva on exacerbations, anthropometry, sweat chloride and safety in children with CF in the Netherlands.
View Article and Find Full Text PDFPediatr Pulmonol
January 2025
Stead Family Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.
Background: People with cystic fibrosis (CF) may not expectorate sputum at young ages or after they receive CFTR modulators. While oropharyngeal swabs are commonly used to test for lower airway pathogens, it is unknown whether Staphylococcus aureus from the oropharynx matches the strain(s) infecting the lungs. Our goal was to determine whether oropharyngeal and sputum isolates of S.
View Article and Find Full Text PDFMonaldi Arch Chest Dis
January 2025
Postgraduate Program in Medical Sciences, State University of Rio de Janeiro; Piquet Carneiro Polyclinic, State University of Rio de Janeiro; Rehabilitation Sciences Postgraduate Program, Augusto Motta University Centre, Rio de Janeiro.
With the increasing use of highly effective modulator therapy (HEMT) in adults with cystic fibrosis (awCF), it is necessary to determine the evolution of the most dynamic physiological markers of this disease, such as the 6-minute walk test (6MWT) and the Glittre-activities of daily living test (TGlittre). The present study aimed to evaluate the 1-year changes in the 6- minute walking distance (6MWD), TGlittre time, and quality of life (QoL) in awCF before the initiation of HEMT and to determine the impact of habitual physical activity (HPA) and chest physiotherapy (CP). This longitudinal study enrolled 24 awCF who completed the 6MWT and TGlittre.
View Article and Find Full Text PDFExpert Rev Respir Med
January 2025
Department of Surgery, Dentistry, Pediatrics and Gynaecology, Pediatric Division, University of Verona, Verona, Italy.
Introduction: Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by the production of sickle hemoglobin, leading to red blood cells sickling and hemolysis in hypoxic conditions. The resulting acute and chronic endothelial inflammation leads to chronic organ damage. Respiratory manifestations in SCD usually start from childhood and represent the leading causes of morbidity and mortality.
View Article and Find Full Text PDFSci Diabetes Self Manag Care
January 2025
Division of Endocrinology and Diabetes, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Purpose: The purpose of the study was to identify the most common reasons for and timing of continuous glucose monitoring (CGM) attrition in youth with type 1 diabetes (T1DM).
Methods: This single center retrospective chart review included youth with T1DM <22 years seen between November 1, 2021, and October 31, 2022. Data were gathered from CGM cloud-based software and the electronic medical record.
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