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Objective: Sinus disease is prevalent in persons with cystic fibrosis (PwCF) and may be a reservoir of airway infection in postlung transplant (pTx) patients. The microbial composition of cystic fibrosis sinuses and its associations with chronic rhinosinusitis (CRS) is relatively unexplored. We aimed to examine the sinus and lower airway microbiome and their associations with CRS in PwCF and pTxPwCF.
Study Design: Prospective single-centre study.
Setting: A total of 31 sex and age (±2 years) matched PwCF and pTxPwCF.
Methods: Demographic and clinical data along with sinus swabs and sputum were collected. CRS was assessed using Sinonasal Outcome Test-22 (SNOT-22) (patient reported outcome) and Lund-McKay (computed tomography sinus) scores. Samples underwent MiSeq Illumina sequencing of the universal 16S ribosomal RNA gene.
Results: A total of 31 PwCF (15 pTxPwCF) were included. Aggregate airways microbiome composition was dominated by (46%), (14%), (11%), (10%), and (6%). α-diversity was significantly lower in post-Tx samples across both sputum and sinus samples ( = .005). β-diversity was significantly different between sputum ( = .004), but not sinus ( = .75) samples by transplant status. While there was a trend in higher β-diversity associated with lower SNOT-22 score at time of first visit, this did not reach significance ( = .05).
Conclusion: Sinus and airway microbiomes differed in PwCF and pTxPwCF, but the prevalent organisms remained consistent. Elucidating the relationship of the microbiome with clinical status to better understand when to intervene accordingly is needed to optimize sinus disease management in PwCF.
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http://dx.doi.org/10.1002/oto2.101 | DOI Listing |
J Cyst Fibros
September 2025
Department of Medicine, Division of Pulmonary, Allergy, and Critical Care Medicine, Oregon Health and Science University, Portland, OR, USA.
Recent improvements in cell-free DNA technology have enabled non-invasive prenatal testing (NIPT) to screen for fetal single-gene autosomal recessive conditions from maternal blood as early as the first trimester. This technique can determine the fetal risk for cystic fibrosis (CF) with a single blood sample from a pregnant person without the need for a partner sample, which is required for traditional carrier screening. A retrospective review of 100,106 consecutive general-risk pregnant patients who underwent CF carrier screening was completed.
View Article and Find Full Text PDFJ Cyst Fibros
September 2025
Division of Paediatric Pulmonology; University of Cape Town, South Africa; Red Cross War Memorial Children's Hospital, Cape Town, South Africa.
Background: Cystic fibrosis (CF) is a genetic disorder that remains underrecognized across Africa, where limited diagnostic capacity, low awareness, and competing health priorities contribute to delayed or missed diagnoses [1-4]. Although increasing data suggests CF is more prevalent than previously believed in Africa, survival remains poor [1]. These challenges do not only affect people with CF (pwCF) in Africa but also have implications for global understanding of the disease, particularly among populations historically excluded from CF research and treatment advances.
View Article and Find Full Text PDFLancet Respir Med
September 2025
Department for Paediatric Pneumology, Allergology, and Neonatology and German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease, Hannover Medical School, 30625 Hannover, Germany. Electronic address:
Lancet Respir Med
September 2025
Effi-Stat, Paris, France.
Background: Among people with cystic fibrosis, sweat chloride and lung function response to elexacaftor-tezacaftor-ivacaftor (ETI) is variable. We hypothesised that the presence of two versus one ETI-responsive CFTR variant could predict response variability.
Methods: In this analysis of two real-world observational studies, data from a French national cohort of adults (aged ≥18 years) with cystic fibrosis and at least one F508del variant treated with ETI and the French compassionate programme for ETI in people (aged ≥6 years) with cystic fibrosis without F508del were used to examine sweat chloride concentrations (SCCs) after ETI initiation, and the absolute change in SCC and percentage of predicted forced expiratory volume in 1 s (ppFEV) following ETI initiation.
Inflamm Bowel Dis
September 2025
Pediatric Gastroenterology, Hepatology and Cystic Fibrosis Unit, Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy.
Background: The effect of sarcopenia on clinical outcomes in children with Crohn's disease (CD) is unknown. We investigated whether sarcopenia at the diagnosis impacts the outcomes of children with CD.
Methods: This was a retrospective, single-center, case-control study of newly diagnosed children with CD undergoing magnetic resonance (MR) within 1 month from the diagnosis, from 2011 to 2022.