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Open research questions in Cystic Fibrosis Research Advances

102 unresolved questions extracted from the limitations and future-work sections of 438 Cystic Fibrosis Research Advances papers in our library. Each links back to the study that raised it.

What the literature leaves open

  • Further research is needed to understand patient perspectives on reproductive genetic counseling, - Studies should investigate the effectiveness of genetic counseling in improving reproductive health outcomes for people with CF

    Assessing the need for reproductive genetic counseling among adults with cystic fibrosis · 2026 · DOI

  • Limited literature documenting patient perspectives on reproductive genetic counseling - Lack of implementation of genetic counseling in CF care teams - Limited understanding of reproductive health concerns among adults with CF

    Assessing the need for reproductive genetic counseling among adults with cystic fibrosis · 2026 · DOI

  • Investigate the clinical significance of microbial contamination in inhalers, - Compare the microbial profiles of different types of inhalers, - Study the effects of microbial contamination on patient outcomes

    Inhaler Devices as Potential Bacterial Reservoirs: A 30-Day Microbiota Analysis in Non-Cystic Fibrosis Bronchiectasis · 2026 · DOI

  • Prior studies have relied exclusively on culture-dependent methods to sample the mouthpiece or canister spray tip of pMDI devices. The specific duration of device use prior to analysis was not clearly defined in previous studies. The study aims to address these gaps by using 16S rRNA sequencing to investigate the microbial profiles of pMDIs and Respimat devices after 30 days of use.

    Inhaler Devices as Potential Bacterial Reservoirs: A 30-Day Microbiota Analysis in Non-Cystic Fibrosis Bronchiectasis · 2026 · DOI

  • Small sample size, - Limited number of hospitalised events, - Study only included patients with high-resolution computed tomography-confirmed BE and at least one PA-positive sputum culture

    Microbiological Factors and Patient-Reported Outcomes of Exacerbation Burden in Pseudomonas aeruginosa-Colonised Bronchiectasis: A One-Year Prospective Study · 2026 · DOI

  • Further studies are needed to investigate the relationship between microbiological characteristics and exacerbation burden, - Research should focus on identifying factors that drive exacerbation risk and hospitalisation in PA-colonised BE patients

    Microbiological Factors and Patient-Reported Outcomes of Exacerbation Burden in Pseudomonas aeruginosa-Colonised Bronchiectasis: A One-Year Prospective Study · 2026 · DOI

  • Study the role of AQP4 in the functioning of the protective mechanisms of the brain during the development of PD and other neurodegenerative diseases - Investigate the interconnection between sleep, AQP4, and glymphatic/lymphatic functions - Examine the effects of AQP4 modulation on neuroprotection

    Aquaporin-4 and Parkinson’s Disease · 2024 · DOI

  • The relationship between AQP4 and alpha-synuclein accumulation is not fully understood. The extent to which the glymphatic system influences the clearance of pathological forms of alpha-synuclein in the human brain is poorly understood.

    Aquaporin-4 and Parkinson’s Disease · 2024 · DOI

  • Elexacaftor/tezacaftor/ivacaftor (ETI) substantially improves clinical outcomes in people with CF (pwCF), but its effects on epigenetic aging and their relationship with inflammation remain poorly understood.

    Elexacaftor/tezacaftor/ivacaftor treatment is associated with epigenetic age deceleration in cystic fibrosis · 2026 · DOI

  • Pathogenic variants in CCDC151 have been reported rarely, and azoospermia associated with CCDC151 -related PCD remains poorly characterized .

    A novel homozygous frameshift mutation in the CCDC151 causing primary ciliary dyskinesia with azoospermia in a Chinese family · 2026 · DOI

  • CFTR gene variants are rare in China, and the pathogenicity of over half of the associated loci remains unclear.

    Clinical and genetic characteristics of diseases caused by CFTR gene mutations in 15 Chinese children: a retrospective analysis · 2025 · DOI

  • Although several CFTR inhibitors have been developed by high-throughput screening, their modes of action remain elusive.

    Structural basis for CFTR inhibition by CFTR inh -172 · 2024 · DOI

  • However, little is known about the long-term predictive value of CGM measures of glycemia for both the development of CFRD and their effect on key clinical outcomes such as weight maintenance and pulmonary function.

    Continuous glucose monitoring and advanced glycation endproducts for prediction of clinical outcomes and development of cystic fibrosis-related diabetes in adults with CF · 2024 · DOI

  • Future studies are needed to investigate CGM as a diagnostic and screening tool for CFRD and to understand the implications of AGE measures in this patient population.

    Continuous glucose monitoring and advanced glycation endproducts for prediction of clinical outcomes and development of cystic fibrosis-related diabetes in adults with CF · 2024 · DOI

  • While competition might often dominate such interactions, little is known about whether bacteria can sense competitors and mount adequate responses.

    RNA-Seq reveals that Pseudomonas aeruginosa mounts growth medium-dependent competitive responses when sensing diffusible cues from Burkholderia cenocepacia · 2024 · DOI

  • The evolution of technology has allowed much of the care provided in person to be replaced by a telehealth delivery model, but studies on telerehabilitation are scarce and dispersed.

    Telerehabilitation in Children and Adolescents with Cystic Fibrosis: A Scoping Review · 2024 · DOI

  • Background In current literature there are only scarce data on the host inflammatory response during Burkholderia cepacia complex (Bcc) persistence.

    Burkholderia cepacia in cystic fibrosis children and adolescents: overall survival and immune alterations · 2024 · DOI

  • influenzae is often associated with pulmonary exacerbations (PEx) in pediatric cohorts, but in adults, studies have yielded conflicting reports around the impact(s) on clinical outcomes such as lung function decline.

    Clinical epidemiology and impact of Haemophilus influenzae airway infections in adults with cystic fibrosis · 2024 · DOI

  • This study has several limitations. First, the relatively small sample size may limit statistical power and the gen- eralizability of the findings. Second, asthma diagnosis was based on a combination of clinical and spirometric assessment; however, inconsistent objective confirma- tion, along with predominantly non-obstructive baseline spirometry and the absence of variability assessment, may have introduced a risk of misclassification and lim- ited diagnostic validity. In addition, the use of bronchodilator reversibility testing in a predominantly non-obstructive population reduces its diagnostic utility, particularly in the absence of consistently reported absolute FEV₁ changes (mL). The cross-sectional design and lack of longitudinal follow- up also limit the ability to assess disease progression and causal relationships. Furthermore, potential recall bias in determining dis- ease chronology and recruitment from a single respira- tory center may introduce selection bias and affect the external validity of the results. The primarily descriptive nature of the analysis, without multivariable adjustment for potential confounders, may limit deeper interpreta- tion of the observed associations. Finally, variability in the application of standardized assessment tools may affect reproducibility.

    Clinical and spirometric characteristics of patients with asthma and bronchiectasis: a comparative observational study · 2026 · DOI

  • Bronchopneumonia is a common lung infection in children, with high morbidity and mortality rates. Nursing care for children with bronchopneumonia can be challenging due to the complexity of the disease. There is a need for more research on nursing care for children with bronchopneumonia.

    Asuhan Keperawatan Anak Dengan Bronkopneumonia Di Ruangan Anak RSUD Prof. H. Muhammad Yamin, S. H Pariaman · 2026 · DOI

  • There is a need for more studies on nursing care for children with bronchopneumonia. There is a lack of research on the effectiveness of comprehensive nursing care in improving the patient's condition.

    Asuhan Keperawatan Anak Dengan Bronkopneumonia Di Ruangan Anak RSUD Prof. H. Muhammad Yamin, S. H Pariaman · 2026 · DOI

  • There is a lack of effective disease monitoring guidelines for cystic fibrosis, particularly in India. There is a need for a more sensitive and reliable assessment of lung disease progression in patients with cystic fibrosis.

    Radiologic progression with minimal functional decline in cystic fibrosis: Insights from a prospective study · 2026 · DOI

  • The exact mechanisms by which CFTR mutations contribute to male infertility are not fully understood. The role of CFTR in early embryonic development is not well established.

    Beyond obstructive azoospermia: emerging insights into reproductive health in men with cystic fibrosis · 2026 · DOI

  • The paper identifies a gap in the understanding of primary ciliary dyskinesia (PCD) and its genetic causes. The study highlights the need for further research on the genetic variants associated with PCD and their pathogenic roles. The authors also identify a gap in the diagnosis of PCD, highlighting the importance of integrating multiple analyses for accurate diagnosis.

    Functional Characterization of a Novel Homozygous DNAH5 Single-Nucleotide Intronic Deletion in a Consanguineous Portuguese Family with Primary Ciliary Dyskinesia · 2026 · DOI

  • There is a need to update the epidemiological trends of cystic fibrosis in Brazil. The demographic profile of the population with cystic fibrosis is not well characterized.

    TENDÊNCIAS EPIDEMIOLÓGICAS DA FIBROSE CÍSTICA NO BRASIL: UMA ANÁLISE DO REGISTRO NACIONAL, 2009-2022 · 2026 · DOI

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102 open questions have been extracted from the limitations and future-work passages of 438 Cystic Fibrosis Research Advances papers in our library. Each one below links back to the study that raised it, so you can read the original claim in context.

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