Reduction in abdominal symptoms (CFAbd-Score), faecal M2-pyruvate-kinase and Calprotectin over one year of treatment with Elexacaftor-Tezacaftor-Ivacaftor in people with CF aged ≥12 years - The RECOVER study.

Background: RECOVER is a multicentre post-approval study of Elexacaftor/Tezacaftor/Ivacaftor (ETI) in pwCF in Ireland and the UK. The CFAbd-Score is the first validated CF-specific patient reported outcome measure (PROM) focusing on gastrointestinal symptoms; it comprises 28 items in 5 domains. In a preliminary study, we previously reported reductions in abdominal symptoms (AS) in pwCF after 26 weeks of ETI-therapy using the CFAbd-Score.

Improvement in Lung Clearance Index and Chest Computed Tomography Scores with Elexacaftor/Tezacaftor/Ivacaftor Treatment in People with Cystic Fibrosis Aged 12 Years and Older - The RECOVER Trial.

Clinical trials have shown that use of elexacaftor/tezacaftor/ivacaftor (ETI) is associated with improvements in sweat chloride, pulmonary function, nutrition, and quality of life in people with cystic fibrosis (CF). Little is known about the impact of ETI on ventilation inhomogeneity and lung structure. RECOVER is a real-world study designed to measure the impact of ETI in people with CF.

Knowledge, attitudes and practices of Irish doctors regarding stop smoking care and electronic cigarettes.

Background: Smoking continues to cause harm on a huge scale in Ireland. Doctors can help this harm through providing safe, effective and clinically sound stop smoking care, but the needs of Irish doctors in this area are largely uncharted.

Aims: We assessed the knowledge, attitudes and practices of Irish doctors regarding stop smoking care and electronic cigarettes.

The clinical impact of Lumacaftor-Ivacaftor on structural lung disease and lung function in children aged 6-11 with cystic fibrosis in a real-world setting.

Background: Data from clinical trials of lumacaftor-ivacaftor (LUM-IVA) demonstrate improvements in lung clearance index (LCI) but not in FEV in children with Cystic Fibrosis (CF) aged 6-11 years and homozygous for the Phe508del mutation. It is not known whether LUM/IVA use in children can impact the progression of structural lung disease. We sought to determine the real-world impact of LUM/IVA on lung structure and function in children aged 6-11 years.

The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey.

Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers.

Defining Age-specific Relationships of Respiratory Syncytial Virus and Rhinovirus Species in Hospitalized Children With Acute Wheeze.

Background: Acute wheezing is one of the most common hospital presentations for young children. Respiratory syncytial virus (RSV) and rhinovirus (RV) species A, B and the more recently described species C are implicated in the majority of these presentations. However, the relative importance and age-specificities of these viruses have not been defined.

Efficacy and adherence of noninvasive ventilation treatment in children with Down syndrome.

Objective: Children with Down syndrome (DS) have an increased prevalence of obstructive sleep apnea (OSA). Noninvasive ventilation (NIV) is a common modality of OSA treatment in this cohort. This study aimed to measure adherence and efficiency of NIV delivery in children with DS.

Sleep disordered breathing in children with Down syndrome in the Republic of Ireland.

Down syndrome (DS) is associated with a complex respiratory phenotype, including obstructive sleep apnea syndrome (OSAS). The study explored parent-reported prevalence and presentation of OSAS symptoms in children and adolescents with DS in Ireland. It also investigated treatment and compliance in those who have OSAS.

A case of COVID-19 in a patient with a univentricular heart post total cavopulmonary connection (Fontan) surgery.

Coronavirus disease 2019 (COVID-19) has caused a global pandemic which has affected patients and healthcare systems around the world. Patients with underlying health conditions seem to be more severely affected. There are limited reports of patients with univentricular circulations and COVID 19; thus, we report a case of COVID-19 in a patient with a univentricular circulation.

Infants without apparent risk factors with aspiration as a cause of respiratory symptoms - a retrospective study.

Background: Dysphagia is common in children born prematurely or those with neuromuscular conditions or airway malformations. Few studies have reported on children with isolated dysphagia and there is significant variation in the literature regarding clinical outcomes.

Aims: The aim of this study was to characterise the clinical presentation of children with isolated dysphagia as well as the diagnostic evaluation, treatment strategies and clinical outcomes.

Neonatal screening programme for CF: Results from the Irish Comparative Outcomes Study (ICOS).

The introduction of NBS in Ireland in July 2011, provided a unique opportunity to investigate clinical outcomes using a comparative historical cohort study. Clinical cohort: children clinically diagnosed with CF born 1 July 2008 to 30 June 2011, and NBS cohort: children diagnosed with CF through NBS born 1 July 2011 to 30 June 2016. Clinical data were collected from the CF Registry of Ireland, medical charts, and data on weight/height before diagnosis from public health nurses and family doctors.

Paediatric asthma deaths in Ireland: 2006-2016.

Background: Asthma is the commonest chronic condition in childhood but mortality from asthma during childhood is a rare occurrence. No national review into asthma deaths in children in Ireland has been performed to date.

Aim: The aim of this study was to review all cases of mortality from asthma in the paediatric population over a 10-year period in Ireland.

Multiorgan involvement and management in children with Down syndrome.

Aim: To review multiorgan involvement and management in children with Down syndrome (DS).

Methods: A literature review of articles from 1980 to 2019 using the MEDLINE interface of PubMed was performed using the following search terms- [Down syndrome] or [Trisomy 21] AND [Cardiology] or [Respiratory] or [neurodevelopment] or [epilepsy] or [musculoskeletal] or [immune system] or [haematological] or [endocrine] or [gastrointestinal] or [ophthalmological] or [Ear Nose Throat] or [dermatology] or [renal].

Results: Congenital heart disease particularly septal defects occur in over 60% of infants with DS and 5%-34% of infants develop persistent pulmonary hypertension of the newborn irrespective of a diagnosis of congenital heart disease.

Viral respiratory infections and the oropharyngeal bacterial microbiota in acutely wheezing children.

Acute viral wheeze in children is a major cause of hospitalisation and a major risk factor for the development of asthma. However, the role of the respiratory tract microbiome in the development of acute wheeze is unclear. To investigate whether severe wheezing episodes in children are associated with bacterial dysbiosis in the respiratory tract, oropharyngeal swabs were collected from 109 children with acute wheezing attending the only tertiary paediatric hospital in Perth, Australia.

Laps or lengths? The effects of different exercise programs on asthma control in children.

Introduction: Exercise training has been shown in numerous studies to improve lung function and asthma control in children with asthma. Swimming has been shown to be of benefit in children with asthma, but which form of exercise is better for asthmatics has not been determined to date. The aim of this study was to examine if swimming improved lung function and asthma symptoms in asthmatic children when compared with different forms of exercise and a control group.

Pulmonary arteriovenous malformations: an uncharacterised phenotype of dyskeratosis congenita and related telomere biology disorders.

Pulmonary arteriovenous malformations are under-recognized in telomere biology disorders and present diagnostic and therapeutic challenges.

A longitudinal study of lung function from 1 month to 18 years of age.

Background: Our hypothesis was that factors associated with wheeze will be associated with changes in lung function trajectory between 1 month and 18 years of age.

Methods: Measurements of lung function were made in individuals aged 1, 6 and 12 months (V'maxFRC), and also at ages 6, 12 and 18 years (FEF(25-75)). Changes in lung function over time relative to sex, a history of asthma, maternal asthma and other factors were explored using random coefficient models.

Comparison of rhinovirus antibody titers in children with asthma exacerbations and species-specific rhinovirus infection.

Background: Asthma exacerbations are associated with human rhinovirus (HRV) infections, and more severe exacerbations are associated with HRV-C. We have previously shown that the HRV-C-specific antibody response is low in healthy adult sera and that most of the antibody to HRV-C is cross-reactive with HRV-A.

Objectives: To compare the antibody response to each HRV species in asthmatic and nonasthmatic children in whom the type of HRV infection was known.

Reduced infant lung function, active smoking, and wheeze in 18-year-old individuals.

Unlabelled: IMPORTANCE This is the first study to link reduced lung function in early life, before the development of symptoms, to wheeze in 18-year-olds. Additionally, the study gives insight into factors other than reduced lung function that are also associated with persistent wheeze in young adults.

Objective: To test the hypothesis that reduced lung function in early life is associated with increased risk for persistent wheeze at age 18 years.