OOIR: Observatory of International Research

Papers

(The H4-Index of Journal of Cystic Fibrosis is 35. 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 2020-11-01 to 2024-11-01.)

Article	Citations
Worldwide rates of diagnosis and effective treatment for cystic fibrosis	163
Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis	85
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)	78
The global impact of SARS-CoV-2 in 181 people with cystic fibrosis	75
Elexacaftor co-potentiates the activity of F508del and gating mutants of CFTR	65
Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study	63
Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation	62
Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip	60
Normal pancreatic function and false-negative CF newborn screen in a child born to a mother taking CFTR modulator therapy during pregnancy	58
Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis	58
Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis	57
Dose adjustments of Elexacaftor/Tezacaftor/Ivacaftor in response to mental health side effects in adults with cystic fibrosis	51
Targeting G542X CFTR nonsense alleles with ELX-02 restores CFTR function in human-derived intestinal organoids	50
PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy	50
The effect of elexacaftor/tezacaftor/ivacaftor (ETI) on glycemia in adults with cystic fibrosis	49
Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis	48
Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis	46
Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series	46
Patient and family perceptions of telehealth as part of the cystic fibrosis care model during COVID-19	46
Elexacaftor/tezacaftor/ivacaftor projected survival and long-term health outcomes in people with cystic fibrosis homozygous for F508del	45
Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI	44
A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus	43
Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients	42
Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study	42
Mental status changes during elexacaftor/tezacaftor / ivacaftor therapy	41

A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of age with cystic fibrosis homozygous	40
Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020	39
A survey of the prevalence, management and outcome of infants with an inconclusive diagnosis following newborn bloodspot screening for cystic fibrosis (CRMS/CFSPID) in six Italian centres	38
The impact of the COVID-19 pandemic on the emotional well-being and home treatment of Belgian patients with cystic fibrosis, including transplanted patients and paediatric patients	38
Inhaled mRNA therapy for treatment of cystic fibrosis: Interim results of a randomized, double‐blind, placebo‐controlled phase 1/2 clinical study	37
Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation	37
The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis	37
Projecting the impact of delayed access to elexacaftor/tezacaftor/ivacaftor for people with Cystic Fibrosis	37
Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis	35
Telehealth use in cystic fibrosis during COVID-19: Association with race, ethnicity, and socioeconomic factors	35