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A randomised crossover trial of tezacaftor-ivacaftor for gut dysfunction in cystic fibrosis with magnetic resonance imaging (MRI) outcomes. (2023)
Journal Article
Ng, C., Dellschaft, N. S., Hoad, C., Marciani, L., Spiller, R., Crooks, C., …Smyth, A. R. (2023). A randomised crossover trial of tezacaftor-ivacaftor for gut dysfunction in cystic fibrosis with magnetic resonance imaging (MRI) outcomes. NIHR Open Research, 3(65), 1-16. https://doi.org/10.3310/nihropenres.13510.1

Background People with cystic fibrosis (CF) can experience recurrent chest infections, pancreatic exocrine insufficiency and gastrointestinal symptoms. New cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs improve lung fun... Read More about A randomised crossover trial of tezacaftor-ivacaftor for gut dysfunction in cystic fibrosis with magnetic resonance imaging (MRI) outcomes..

Complex roles of TGF-β signaling pathways in lung development and bronchopulmonary dysplasia (2023)
Journal Article
Calthorpe, R. J., Poulter, C., Smyth, A. R., Sharkey, D., Bhatt, J., Jenkins, G., & Tatler, A. L. (2023). Complex roles of TGF-β signaling pathways in lung development and bronchopulmonary dysplasia. AJP - Lung Cellular and Molecular Physiology, 324(3), L285-L296. https://doi.org/10.1152/ajplung.00106.2021

As survival of extremely preterm infants continues to improve, there is also an associated increase in bronchopulmonary dysplasia (BPD), one of the most significant complications of preterm birth. BPD development is multifactorial resulting from expo... Read More about Complex roles of TGF-β signaling pathways in lung development and bronchopulmonary dysplasia.

Childhood Brain Tumors: A Review of Strategies to Translate CNS Drug Delivery to Clinical Trials (2023)
Journal Article
Rahman, R., Janowski, M., Killick-Cole, C. L., Singleton, W. G. B., Campbell, E., Walczak, P., …Walker, D. A. (2023). Childhood Brain Tumors: A Review of Strategies to Translate CNS Drug Delivery to Clinical Trials. Cancers, 15(3), Article 857. https://doi.org/10.3390/cancers15030857

Brain and spinal tumors affect 1 in 1000 people by 25 years of age, and have diverse histological, biological, anatomical and dissemination characteristics. A mortality of 30–40% means the majority are cured, although two-thirds have life-long disabi... Read More about Childhood Brain Tumors: A Review of Strategies to Translate CNS Drug Delivery to Clinical Trials.

Identifying new biomarkers of aggressive Group 3 and SHH medulloblastoma using 3D hydrogel models, single cell RNA sequencing and 3D OrbiSIMS imaging (2023)
Journal Article
Linke, F., Johnson, J. E. C., Kern, S., Bennett, C. D., Lourdusamy, A., Lea, D., …Coyle, B. (2023). Identifying new biomarkers of aggressive Group 3 and SHH medulloblastoma using 3D hydrogel models, single cell RNA sequencing and 3D OrbiSIMS imaging. Acta Neuropathologica Communications, 11(1), Article 6. https://doi.org/10.1186/s40478-022-01496-4

The most common malignant brain tumour in children, medulloblastoma (MB), is subdivided into four clinically relevant molecular subgroups, although targeted therapy options informed by understanding of different cellular features are lacking. Here, b... Read More about Identifying new biomarkers of aggressive Group 3 and SHH medulloblastoma using 3D hydrogel models, single cell RNA sequencing and 3D OrbiSIMS imaging.

Bronchopulmonary dysplasia prediction models: a systematic review and meta-analysis with validation (2023)
Journal Article
Kwok, T. C., Batey, N., Luu, K. L., Prayle, A., & Sharkey, D. (2023). Bronchopulmonary dysplasia prediction models: a systematic review and meta-analysis with validation. Pediatric Research, 94, 43–54. https://doi.org/10.1038/s41390-022-02451-8

Abstract: Prediction models could identify infants at the greatest risk of bronchopulmonary dysplasia (BPD) and allow targeted preventative strategies. We performed a systematic review and meta-analysis with external validation of identified models.... Read More about Bronchopulmonary dysplasia prediction models: a systematic review and meta-analysis with validation.

Prebiotics for people with cystic fibrosis (2022)
Journal Article
Williams, N., Jayaratnasingam, J., Prayle, A. P., Nevitt, S. J., & Smyth, A. R. (2022). Prebiotics for people with cystic fibrosis. Cochrane Database of Systematic Reviews, 2022(12), Article CD015236. https://doi.org/10.1002/14651858.cd015236

Background. Description of the condition. Cystic fibrosis (CF) is a life‐limiting genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It affects approximately 100,000 children and adults worldwi... Read More about Prebiotics for people with cystic fibrosis.

Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis (2022)
Journal Article
Smyth, A. R., Lo, D. K., & Muhlebach, M. S. (2022). Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis. Cochrane Database of Systematic Reviews, 2022(12), Article CD009650. https://doi.org/10.1002/14651858.cd009650.pub5

Background Cystic fibrosis is an inherited recessive disorder of chloride transport that is characterised by recurrent and persistent pulmonary infections from resistant organisms that result in lung function deterioration and early mortality in suf... Read More about Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis.

Development of nanoparticle loaded microneedles for drug delivery to a brain tumour resection site (2022)
Journal Article
Muresan, P., McCrorie, P., Smith, F., Vasey, C., Taresco, V., Scurr, D. J., …Marlow, M. (2023). Development of nanoparticle loaded microneedles for drug delivery to a brain tumour resection site. European Journal of Pharmaceutics and Biopharmaceutics, 182, 53-61. https://doi.org/10.1016/j.ejpb.2022.11.016

Systemic drug delivery to the central nervous system (CNS) has been historically impeded by the presence of the blood brain barrier rendering many therapies inefficacious to any cancer cells residing within the brain. Therefore, local drug delivery s... Read More about Development of nanoparticle loaded microneedles for drug delivery to a brain tumour resection site.

Histopathological impact of Redox-responsive methacrylamide based micellar nanoparticles on Orthotopic Models of Triple Negative Breast Cancers (2022)
Journal Article
Mehradnia, F., Moloney, C., Cavanagh, R., Pearce, A. K., Ritchie, A., Clarke, P., …Alexander, C. (2022). Histopathological impact of Redox-responsive methacrylamide based micellar nanoparticles on Orthotopic Models of Triple Negative Breast Cancers. British Journal of Pharmacy, 7(2), https://doi.org/10.5920/bjpharm.1173

The therapeutic efficacy of anticancer nanocarriers ishighly dependent on their size, shape, targeting ability, and stimuli-responsiveness. Herein, we studied the in vivo therapeutic efficacy ofDoxorubicin (Dox) loaded redox responsive micellar-like... Read More about Histopathological impact of Redox-responsive methacrylamide based micellar nanoparticles on Orthotopic Models of Triple Negative Breast Cancers.

Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis (2022)
Journal Article
Southern, K. W., Castellani, C., Lammertyn, E., Smyth, A., VanDevanter, D., van Koningsbruggen-Rietschel, S., …Duff, A. (2023). Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis. Journal of Cystic Fibrosis, 22(1), 17-30. https://doi.org/10.1016/j.jcf.2022.10.002

Cystic fibrosis (CF) has entered the era of variant-specific therapy, tailored to the genetic variants in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CFTR modulators, the first variant-specific therapy available, have transfo... Read More about Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis.

Electric field responsive nanotransducers for glioblastoma (2022)
Journal Article
Jain, A., Jobson, I., Griffin, M., Rahman, R., Smith, S., & Rawson, F. J. (2022). Electric field responsive nanotransducers for glioblastoma. Bioelectronic Medicine, 8, Article 17. https://doi.org/10.1186/s42234-022-00099-7

Background Electric field therapies such as Tumor Treating Fields (TTFields) have emerged as a bioelectronic treatment for isocitrate dehydrogenase wild-type and IDH mutant grade 4 astrocytoma Glioblastoma (GBM). TTFields rely on alternating curre... Read More about Electric field responsive nanotransducers for glioblastoma.

Spatial transcriptomic analysis delineates epithelial and mesenchymal subpopulations and transition stages in childhood ependymoma (2022)
Journal Article
Fu, R., Norris, G. A., Willard, N., Griesinger, A. M., Riemondy, K. A., Amani, V., …Donson, A. M. (2023). Spatial transcriptomic analysis delineates epithelial and mesenchymal subpopulations and transition stages in childhood ependymoma. Neuro-Oncology, 25(4), 786-798. https://doi.org/10.1093/neuonc/noac219

Background The diverse cellular constituents of childhood brain tumor ependymoma, recently revealed by single cell RNA-sequencing, may underly therapeutic resistance. Here we use spatial transcriptomics to further advance our understanding of the tum... Read More about Spatial transcriptomic analysis delineates epithelial and mesenchymal subpopulations and transition stages in childhood ependymoma.

Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis (2022)
Journal Article
McLeod, C., Smyth, A. R., Messer, M., Schultz, A., Wood, J., Norman, R., …Snelling, T. L. (2022). Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis. BMJ Open, 12(9), Article e056528. https://doi.org/10.1136/bmjopen-2021-056528

INTRODUCTION: Pulmonary exacerbations are associated with increased morbidity and mortality in people with cystic fibrosis (CF). There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations or how these outcome... Read More about Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis.

Parental Programming of Offspring Health: The Intricate Interplay between Diet, Environment, Reproduction and Development (2022)
Journal Article
Batra, V., Norman, E., Morgan, H. L., & Watkins, A. J. (2022). Parental Programming of Offspring Health: The Intricate Interplay between Diet, Environment, Reproduction and Development. Biomolecules, 12(9), Article 1289. https://doi.org/10.3390/biom12091289

As adults, our health can be influenced by a range of lifestyle and environmental factors, increasing the risk for developing a series of non-communicable diseases such as type 2 diabetes, heart disease and obesity. Over the past few decades, our und... Read More about Parental Programming of Offspring Health: The Intricate Interplay between Diet, Environment, Reproduction and Development.

Neonatal stroke surveillance study protocol in the United Kingdom and Republic of Ireland (2022)
Journal Article
Kwok, T. C., Dineen, R. A., Whitehouse, W., Lynn, R. M., McSweeney, N., & Sharkey, D. (2022). Neonatal stroke surveillance study protocol in the United Kingdom and Republic of Ireland. Open Medicine, 17(1), 1417-1424. https://doi.org/10.1515/med-2022-0554

Neonatal stroke is a devastating condition that causes brain injury in babies and often leads to lifelong neurological impairment. Recent prospective population studies of neonatal stroke are lacking. Neonatal strokes are different from those in olde... Read More about Neonatal stroke surveillance study protocol in the United Kingdom and Republic of Ireland.

Modelling Variations in Newborn Life Support Procedure Using Colored Petri Nets (2022)
Conference Proceeding
Leva, M. C., Patelli, E., Podofillini, L., Wilson, S., Tan, A., Remenyte-Prescott, R., …Sharkey, D. (2022). Modelling Variations in Newborn Life Support Procedure Using Colored Petri Nets. In M. Chiara Leva, E. Patelli, L. Podofillini, & S. Wilson (Eds.), Proceedings of the 32nd European Safety and Reliability Conference (ESREL 2022) (489-496). https://doi.org/10.3850/978-981-18-5183-4_R12-08-152-cd

This research is conducted to study variations in the Newborn Life Support procedure. This procedure follows an evidence-based protocol to resuscitate and stabilize newborn babies requiring assistance at birth. Errors and ineffective actions in this... Read More about Modelling Variations in Newborn Life Support Procedure Using Colored Petri Nets.

Impact of Tumour Epstein–Barr Virus Status on Clinical Outcome in Patients with Classical Hodgkin Lymphoma (cHL): A Review of the Literature and Analysis of a Clinical Trial Cohort of Children with cHL (2022)
Journal Article
Nohtani, M., Vrzalikova, K., Ibrahim, M., Powell, J. E., Fennell, É., Morgan, S., …Murray, P. G. (2022). Impact of Tumour Epstein–Barr Virus Status on Clinical Outcome in Patients with Classical Hodgkin Lymphoma (cHL): A Review of the Literature and Analysis of a Clinical Trial Cohort of Children with cHL. Cancers, 14(17), Article 4297. https://doi.org/10.3390/cancers14174297

In this study, we have re-evaluated how EBV status influences clinical outcome. To accomplish this, we performed a literature review of all studies that have reported the effect of EBV status on patient outcome and also explored the effect of EBV pos... Read More about Impact of Tumour Epstein–Barr Virus Status on Clinical Outcome in Patients with Classical Hodgkin Lymphoma (cHL): A Review of the Literature and Analysis of a Clinical Trial Cohort of Children with cHL.

Lipoprotein Deprivation Reveals a Cholesterol-Dependent Therapeutic Vulnerability in Diffuse Glioma Metabolism (2022)
Journal Article
Wood, J., Abdelrazig, S., Evseev, S., Ortori, C., Castellanos-Uribe, M., May, S. T., …Rahman, R. (2022). Lipoprotein Deprivation Reveals a Cholesterol-Dependent Therapeutic Vulnerability in Diffuse Glioma Metabolism. Cancers, 14(16), Article 3873. https://doi.org/10.3390/cancers14163873

Poor outcomes associated with diffuse high-grade gliomas occur in both adults and children, despite substantial progress made in the molecular characterisation of the disease. Targeting the metabolic requirements of cancer cells represents an alterna... Read More about Lipoprotein Deprivation Reveals a Cholesterol-Dependent Therapeutic Vulnerability in Diffuse Glioma Metabolism.

Oxygen-Tolerant RAFT Polymerization Initiated by Living Bacteria (2022)
Journal Article
Gurnani, P., Bennett, M. R., Moloney, C., Catrambone, F., Turco, F., Myers, B., …Rawson, F. J. (2022). Oxygen-Tolerant RAFT Polymerization Initiated by Living Bacteria. ACS Macro Letters, 11(8), 954-960. https://doi.org/10.1021/acsmacrolett.2c00372

Living organisms can synthesize a wide range of macromolecules from a small set of natural building blocks, yet there is potential for even greater materials diversity by exploiting biochemical processes to convert unnatural feedstocks into new abiot... Read More about Oxygen-Tolerant RAFT Polymerization Initiated by Living Bacteria.

Oxygen-Tolerant RAFT Polymerization Initiated by Living Bacteria (2022)
Journal Article
Bennett, M. R., Moloney, C., Catrambone, F., Turco, F., Myers, B., Kovacs, K., …Gurnani, P. (2022). Oxygen-Tolerant RAFT Polymerization Initiated by Living Bacteria. ACS Macro Letters, 11(8), 954-960. https://doi.org/10.1021/acsmacrolett.2c00372

Living organisms can synthesize a wide range of macromolecules from a small set of natural building blocks, yet there is potential for even greater materials diversity by exploiting biochemical processes to convert unnatural feedstocks into new abiot... Read More about Oxygen-Tolerant RAFT Polymerization Initiated by Living Bacteria.