OOIR: Observatory of International Research

Papers

(The H4-Index of Journal of Cystic Fibrosis is 37. The table below lists those papers that are above that threshold based on CrossRef citation counts [max. 250 papers]. The publications cover those that have been published in the past four years, i.e., from 2022-08-01 to 2026-08-01.)

Article	Citations
564 “How do you live the best life you can with this pain?” A qualitative study of the challenges of living with CF and pain in the modulator era	150
209 Inositol-requiring enzyme 1β is a therapeutic target for cystic fibrosis airway muco-obstructive disease	147
235 Differential regulation of immune function by highly effective CFTR modulators in THP-1-derived macrophages	133
184 Bioengineered three-dimensional cystic fibrosis lung airway model for drug testing	109
808 Implementation of physical therapy and exercise education in annual cystic fibrosis clinic	93
260 RCT2100 rescue of cystic fibrosis (CF) transmembrane conductance regulator function in CF human bronchial epithelial cells and mucociliary clearance in CF ferrets	87
39 Using mouse models to understand the critical roles of granulocyte macrophage colony stimulating factor and macrophages for control of pulmonary Mycobacterium abscessus infection	85
365 Differential effects of (S)- and (R)-vanzacaftor on BKCa potentiation and CFTR correction	83
99 Development and validation of a deep neural network to predict antimicrobial resistance in people with cystic fibrosis using routinely collected electronic healthcare record data	83
27 Standing up to the “bully”: Staphylococcus aureus can sustain long-term infection in the presence of Pseudomonas aeruginosa and may outcompete in the cystic fibrosis airway	80
490 What are the experiences of cystic fibrosis clinical nurse specialists? Are systemic practice ideas unknowingly incorporated when working with children and families?	73
304 Assessing a novel dual-endonuclease system in restoring CFTR expression in cystic fibrosis humanized mouse and human airway models	68
320 Gain-of-function CFTR mutation improves function of W1282X CFTR	67
526 Non-surgical management of urinary incontinence in women with cystic fibrosis	63
558 Challenges faced by parents of children with cystic fibrosis in Ireland: The Irish Comparative Outcome Study of Cystic Fibrosis	62
529 Are adults with cystic fibrosis choosing to continue with airway clearance therapy in the era of highly effective gene modulator therapy?	61
644 Timely initiation of pancreatic enzyme replacement therapy for surgical cystic fibrosis infants in the neonatal intensive care unit	57
773 A quality improvement project to improve the evaluation of low bone density in people with cystic fibrosis	55
586 Description of a novel therapeutic support group for siblings of children with cystic fibrosis: Siblings Supporting Others and Achieving Resilience	55
Restoring airway epithelial homeostasis in Cystic Fibrosis	53
WS13.01 The cystic fibrosis Home Sputum-Induction Trial (CF-HomeSpIT - ISRCTN86523335) to evaluate home sputum-induction and early morning saliva sampling in children with cystic fibrosis	53
Autoimmunity in people with cystic fibrosis	52
78 Pseudomonas aeruginosa induced GM-CSF production by airway epithelial cells could help protect against Mycobacterium abscessus	51
325 Evaluation of CFTR gene editing efficacy using patient-derived nose- on-chip model	48
227 Nascent chain misfolding slows translation and stimulates co- translational mRNA decay	47

203 Airway dysanapsis is associated with residual pulmonary exacerbation risk in children with cystic fibrosis treated with elexacaftor/tezacaftor/ ivacaftor	46
346 Leaky gut, dysbiosis, and inflammation support inter-organ communication in cystic fibrosis	44
97 Separability of membrane permeabilization and cell killing activities of the human host defense peptide LL-37 in Pseudomonas aeruginosa	44
748 Development of metabolic syndrome in people with cystic fibrosis after three years of elexacaftor/tezacaftor/ivacaftor	42
258 Functional correction of CFTR-G542X in human bronchial epithelial cells using a Type I CRISPR adenine base editor with a guide-length- tunable editing window	41
523 Navigating school with cystic fibrosis: perspectives of people with CF and their caregivers	40
41 Loss of MexEF-OprN function increases virulence of clinical Pseudomonas aeruginosa isolates	40
729 Impact of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on healthcare resource utilization (HCRU) over three years in LONGITUDE – a UK CF Registry observational study	39
634 Evaluating shared decision making in lung transplant discussions between clinicians and people with severe CF	39
757 The impacts of highly effective modulator therapy and telehealth on cystic fibrosis care: Changes in visit frequency	38
379 Identifying and prioritizing burdens of cystic fibrosis care for Canadians living with CF	38
241 Functional characterization of an ex vivo ferret model as a precursor for cell therapy	37
475 Physiologically based pharmacokinetic of elexacaftor/tezacaftor/ ivacaftor in children with cystic fibrosis: extrapolation of the dosing regimen in children under 2 years of age	37