ERS International Congress 2023: highlights from the Paediatrics Assembly.

Respiratory health in children is essential for general wellbeing and healthy development in the short and long term. It is well known that many respiratory diseases in adulthood have their origins in early life, and therefore research on prevention of respiratory diseases and management of children with respiratory diseases will benefit patients during the full life course. Scientific and clinical advances in the field of respiratory health are moving at a fast pace. This article summarises some of the highlights in paediatric respiratory medicine presented at the hybrid European Respiratory Society (ERS) International Congress 2023 which took place in Milan (Italy). Selected sessions are summarised by Early Career Members of the Paediatrics Assembly (Assembly 7) under the supervision of senior ERS officers, and cover a wide range of research areas in children, including respiratory physiology and sleep, asthma and allergy, cystic fibrosis, respiratory infection and immunology, neonatology and intensive care, respiratory epidemiology and bronchology.

The Exeter Activity Unlimited statement on physical activity and exercise for cystic fibrosis: methodology and results of an international, multidisciplinary, evidence-driven expert consensus.

BACKGROUND: The roles of physical activity (PA) and exercise within the management of cystic fibrosis (CF) are recognised by their inclusion in numerous standards of care and treatment guidelines. However, information is brief, and both PA and exercise as multi-faceted behaviours require extensive stakeholder input when developing and promoting such guidelines. METHOD: On 30th June and 1st July 2021, 39 stakeholders from 11 countries, including researchers, healthcare professionals and patients participated in a virtual conference to agree an evidence-based and informed expert consensus about PA and exercise for people with CF. This consensus presents the agreement across six themes: (i) patient and system centred outcomes, (ii) health benefits, iii) measurement, (iv) prescription, (v) clinical considerations, and (vi) future directions. The consensus was achieved by a stepwise process, involving: (i) written evidence-based synopses; (ii) peer critique of synopses; (iii) oral presentation to consensus group and peer challenge of revised synopses; and (iv) anonymous voting on final proposed synopses for adoption to the consensus statement. RESULTS: The final consensus document includes 24 statements which surpassed the consensus threshold (>80% agreement) out of 30 proposed statements. CONCLUSION: This consensus can be used to support health promotion by relevant stakeholders for people with CF.

Effects of a Long-Term Wearable Activity Tracker-Based Exercise Intervention on Cardiac Morphology and Function of Patients with Cystic Fibrosis.

Several studies have shown that patients with cystic fibrosis (CF), even at a young age, have pulmonary and cardiac abnormalities. The main complications are cardiac right ventricular (RV) systolic and/or diastolic dysfunction and pulmonary hypertension, which affects their prognosis. Exercise training (ET) is recommended in patients with CF as a therapeutic modality to improve physical fitness and health-related quality of life. However, questions remain regarding its optimal effective and safe dose and its effects on the patients' cardiac function. The study aimed to provide a wearable activity tracker (WAT)-based ET to promote physical activity in CF patients and assess its effects on cardiac morphology and function. Forty-two stable CF individuals (aged 16.8 ± 3.6 years) were randomly assigned to either the intervention (Group A) or the control group (Group B). Group A participated in a 1-year WAT-based ET program three times per week. All patients underwent a 6-min walking test (6-MWT) and an echocardiographic assessment focused mainly on RV anatomy and function at the baseline and the end of the study. RV systolic function was evaluated by measuring the tricuspid annular plane systolic excursion (TAPSE), the systolic tricuspid annular velocity (TVS'), the RV free-wall longitudinal strain (RVFWSL), and the right ventricular four-chamber longitudinal strain (RV4CSL). RV diastolic function was assessed using early (TVE) and late (TVA) diastolic transtricuspid flow velocity and their ratio TVE/A. Pulmonary artery systolic pressure (PASP) was also estimated. In Group A after ET, the 6MWT distance improved by 20.6% (p < 0.05), TVA decreased by 17% (p < 0.05), and TVE/A increased by 13.2% (p < 0.05). Moreover, TAPSE, TVS', RVFWSL, and RV4CSL increased by 8.3% (p < 0.05), 9.0% (p < 0.05), 13.7% (p < 0.05), and 26.7% (p < 0.05), respectively, while PASP decreased by 7.6% (p < 0.05). At the end of the study, there was a significant linear correlation between the number of steps and the PASP (r = −0.727, p < 0.01) as well as the indices of RV systolic function in Group A. In conclusion, WAT is a valuable tool for implementing an effective ET program in CF. Furthermore, ET has a positive effect on RV systolic and diastolic function.

Change in CF care during COVID-19 pandemic: Single-center experience in a middle-income setting.

INTRODUCTION: The coronavirus 2019 (COVID-19) pandemic has demanded care changes for patients with chronic disease. Patients with cystic fibrosis (CF) are considered at higher risk of developing severe manifestations in the case of SARS-CoV-2 infection, and a need for new ways of safer care delivery has been required to avoid transmission. OBJECTIVES: To assess the impact of the lockdown during the first wave of the COVID-19 pandemic and remote monitoring on patient's health status and daily maintenance therapy in a middle-income resource setting. METHODS: During the first wave of the pandemic period, we changed from regular clinic visits to telephone visit calls to monitor our patients' health condition and adherence to physiotherapy and physical exercise. RESULTS: A total of 120 patients or their caregivers have been contacted by telephone call visits over 10 weeks. During this period, 38 patients (28.33%) were identified to have pulmonary exacerbation; 89.5% were prescribed oral antibiotics, 3% were hospitalized to get iv antibiotics, and 8% of the patients presented other CF complications. Most of the patients did not change the frequency of the daily physiotherapy. Moreover, 71% of the patients who performed regular physical exercise changed the frequency and the type of exercise during the quarantine period. Interestingly, mean forced expiratory volume in 1 s and body weight increased significantly and after the lockdown period. CONCLUSIONS: During the COVID-19 pandemic, the implementation of telephone contact processes aiming for CF patients' appropriate care is of great importance. Further studies are needed to evaluate patient outcomes when transitioning from face-to-face clinics to telemedicine clinics.

The Fitter the Better? Cardiopulmonary Exercise Testing Can Predict Pulmonary Exacerbations in Cystic Fibrosis.

BACKGROUND: The role of cardiopulmonary exercise testing (CPET) in the assessment of prognosis in CF (cystic fibrosis) is crucial. However, as the overall survival of the disease becomes better, the need for examinations that can predict pulmonary exacerbations (PEx) and subsequent deterioration becomes evident. METHODS: Data from a 10-year follow up with CPET and spirometry of CF patients were used to evaluate whether CPET-derived parameters can be used as prognostic indexes for pulmonary exacerbations in patients with CF. Pulmonary exacerbations were recorded. We used a survival analysis through Cox Regression to assess the prognostic role of CPET parameters for PeX. CPET parameters and other variables such as sputum culture, age, and spirometry measurements were tested via multivariate cox models. RESULTS: During a 10-year period (2009-2019), 78 CF patients underwent CPET. Cox regression analysis revealed that VO2peak% (peak Oxygen Uptake predicted %) predicted (hazard ratio (HR), 0.988 (0.975, 1.000) p = 0.042) and PetCO2 (end-tidal CO2 at peak exercise) (HR 0.948 (0.913, 0.984) p = 0.005), while VE/VO2 and (respiratory equivalent for oxygen at peak exercise) (HR 1.032 (1.003, 1.062) p = 0.033) were significant predictors of pulmonary exacerbations in the short term after the CPET. Additionally, patients with VO2peak% predicted <60% had 4.5-times higher relative risk of having a PEx than those with higher exercise capacity. CONCLUSIONS: CPET can provide valuable information regarding upcoming pulmonary exacerbation in CF. Patients with VO2peak <60% are at great risk of subsequent deterioration. Regular follow up of CF patients with exercise testing can highlight their clinical image and direct therapeutic interventions.

ERS International Congress 2020: highlights from the Paediatric Assembly.

In this review, the Paediatric Assembly of the European Respiratory Society (ERS) presents a summary of the highlights and most relevant findings in the field of paediatric respiratory medicine presented at the virtual ERS International Congress 2020. Early Career Members of the ERS and Chairs of the different Groups comprising the Paediatric Assembly discuss a selection of the presented research. These cover a wide range of research areas, including respiratory physiology and sleep, asthma and allergy, cystic fibrosis, respiratory infection and immunology, neonatology and intensive care, epidemiology, bronchology and lung and airway development. Specifically, we describe the long-term effect in lung function of premature birth, mode of delivery and chronic respiratory conditions such as cystic fibrosis. In paediatric asthma, we present risk factors, phenotypes and their progression with age, and the challenges in diagnosis. We confirm the value of the lung clearance index to detect early lung changes in cystic fibrosis. For bronchiectasis treatment, we highlight the importance of identifying treatable traits. The use of biomarkers and genotypes to identify infants at risk of long-term respiratory morbidity is also discussed. We present the long-term impact on respiratory health of early life and fetal exposures to maternal obesity and intrauterine hypoxia, mechanical ventilation hyperoxia, aeroallergens, air pollution, vitamin A deficient intake and bronchitis. Moreover, we report on the use of metabolomics and genetic analysis to understand the effect of these exposures on lung growth and alveolar development. Finally, we stress the need to establish multidisciplinary teams to treat complex airway pathologies.

Toward the Establishment of New Clinical Endpoints for Cystic Fibrosis: The Role of Lung Clearance Index and Cardiopulmonary Exercise Testing.

As Cystic Fibrosis (CF) treatment advances, research evidence has highlighted the value and applicability of Lung Clearance Index and Cardiopulmonary Exercise Testing as endpoints for clinical trials. In the context of these new endpoints for CF trials, we have explored the use of these two test outcomes for routine CF care. In this review we have presented the use of these methods in assessing disease severity, disease progression, and the efficacy of new interventions with considerations for future research.

ERS statement on standardisation of cardiopulmonary exercise testing in chronic lung diseases.

The objective of this document was to standardise published cardiopulmonary exercise testing (CPET) protocols for improved interpretation in clinical settings and multicentre research projects. This document: 1) summarises the protocols and procedures used in published studies focusing on incremental CPET in chronic lung conditions; 2) presents standard incremental protocols for CPET on a stationary cycle ergometer and a treadmill; and 3) provides patients' perspectives on CPET obtained through an online survey supported by the European Lung Foundation. We systematically reviewed published studies obtained from EMBASE, Medline, Scopus, Web of Science and the Cochrane Library from inception to January 2017. Of 7914 identified studies, 595 studies with 26 523 subjects were included. The literature supports a test protocol with a resting phase lasting at least 3 min, a 3-min unloaded phase, and an 8- to 12-min incremental phase with work rate increased linearly at least every minute, followed by a recovery phase of at least 2-3 min. Patients responding to the survey (n=295) perceived CPET as highly beneficial for their diagnostic assessment and informed the Task Force consensus. Future research should focus on the individualised estimation of optimal work rate increments across different lung diseases, and the collection of robust normative data.

Ventilation efficiency to exercise in patients with cystic fibrosis.

INTRODUCTION: Exercise ventilation efficiency index in cardiopulmonary exercise testing (CPET) is elevated in patients with heart failure providing useful information on disease progression and prognosis. Few data, however, exist for ventilation efficiency index among cystic fibrosis (CF) patients. AIMS: To assess ventilation efficiency index (ΔVE/ΔVCO2 or V'E/V'CO2 slope) and intercept of ventilation (VE-intercept) in CF patients with mild, moderate, and severe cystic fibrosis (CF) lung disease. To assess possible correlations with ventilation inhomogeneity and structural damages as seen on high resolution computed tomography (HRCT). METHODS: CF patients with mild (FEV1 > 80%, n = 47), moderate (60% < FEV1 < 80%, n = 21), and severe (FEV1 < 60%, n = 9) lung disease, mean age 14.9 years participated. Peak oxygen uptake (VO2 peak), pulmonary ventilation at peak exercise (VE), respiratory equivalent ratios for oxygen and carbon dioxide at peak exercise (VE/VO2 , VE/VCO2 ), end-tidal CO2 (PetCO2 ), and ΔVE/ΔVCO2 , ΔVE/ΔVO2 in a maximal CPET along with spirometry and multiple breath washout indices were examined. HRCT scans were performed and scored using Bhalla score. RESULTS: Mean ΔVE/ΔVCO2 showed no significant differences among the three groups (P = .503). Mean VEint discriminated significantly among the different groups (p 2 < 0.001). Ventilation efficiency index did not correlate either with LCI or Bhalla score. However, VE together with ΔVE/ΔVCO2 slope could predict Bhalla score (r 2 = 0.869, P = .006). CONCLUSION: No significant differences were found regarding ΔVE/ΔVCO2 slope levels between the three groups. Ventilation intercept (VEint ) was elevated significantly as disease progresses reflecting increased dead space ventilation. CF patients retain their ventilation efficiency to exercise even as lung function deteriorates by adopting a higher respiratory rate along with increased dead space ventilation.

Child Morbidity and Disease Burden in Refugee Camps in Mainland Greece.

The crisis conflicts in Syria have forced a lot of people to relocate and live in mainland Greece, where they are hosted in refugee camps. In the present study, our aim was to assess child morbidity and overall disease burden in two camps in northern Greece during a six-month winter period. A primary health care office was founded in each camp. Refugees of all ages with health problems were examined daily by specialty doctors. Cases were classified into two categories: Infectious or non-infectious. In total, 2631 patients were examined during this period (out of the 3760 refugees hosted). Of these patients, 9.8% were infants, 12.7% were toddlers, and 13.4% were children. Most of the visits for children aged less than 12 years old were due to infectious diseases (80.8%). The most common sites of communicable diseases among children were the respiratory tract (66.8%), the skin (23.2%), and the urinary (3.2%) and gastrointestinal tracts (6.2%). Non-communicable diseases were mostly due to gastrointestinal (20.2%), respiratory (18.2%), surgical (13.1%), and allergic (10.3%) disorders. Infants, toddlers, and children suffered more frequently from respiratory infections, while in adolescents and adults, non-infectious diseases were more common. Toddlers and children were more likely to fall ill in comparison to infants. Conclusions: During the winter period, infectious diseases, especially of the respiratory tract, are the main reason for care seeking among refugees in Greek camps, with toddlers suffering more than other age groups. The overall mortality and referral percentage were low, indicating that adequate primary care is provided in this newly established refugee hosting model.

Does Pseudomonas aeruginosa Colonization Affect Exercise Capacity in CF?

INTRODUCTION: Cardio-Pulmonary Exercise Testing (CPET) has been recognized as a valuable method in assessing disease burden and exercise capacity among CF patients. AIM: To evaluate whether Pseudomonas aeruginosa colonization status affects Exercise Capacity, LCI and High-Resolution Computed Tomography (HRCT) indices among patients with CF; to check if Pseudomonas colonization can predict exercise intolerance. SUBJECTS: Seventy-eight (78) children and adults with CF (31 males) mean (range) age 17.08 (6.75; 24.25) performed spirometry, Multiple Breath Washout (MBW) and CPET along with HRCT on the same day during their admission or follow up visit. RESULTS: 78 CF patients (mean FEV1: 83.3% mean LCI: 10.9 and mean VO2 peak: 79.1%) were evaluated: 33 were chronically colonized with Pseudomonas aeruginosa, 24 were intermittently colonized whereas 21 were Pseudomonas free. Statistically significant differences were observed among the three groups in: peak oxygen uptake % predicted (VO2 peak% (p < 0.001), LCI (p < 0.001), as well as FEV1% (p < 0.001) and FVC% (p < 0.001). Pseudomonas colonization could predict VO2 peak% (p < 0.001, r 2: -0.395). CONCLUSION: Exercise capacity as reflected by peak oxygen uptake is reduced in Pseudomonas colonized patients and reflects lung structural damages as shown on HRCT. Pseudomonas colonization could predict exercise limitation among CF patients.

Lung clearance index (LCI) as a predictor of exercise limitation among CF patients.

INTRODUCTION: FEV1 is often considered the gold standard to monitor lung disease in cystic fibrosis (CF). Recently, there has been increasing interest in multiple breath washout (MBW) and cardiopulmonary exercise testing (CPET) as alternative or even more sensitive techniques. However, limited data exist on associations among the above methods. AIM: To evaluate the correlations between outcome measures of MBW and CPET and to examine if ventilation inhomogeneity can predict exercise intolerance. SUBJECTS AND METHODS: Ninety-seven children and adults with CF (47 males, mean [range] age 14.9 (6.6; 26.7) years, mean FEV1 : 90.8% predicted, mean lung clearance index [LCI]: 11.4, and mean peak oxygen uptake [VO2 peak]: 82.4% predicted) performed spirometry, MBW, and CPET on the same day during their admission or outpatient visit. RESULTS: LCI, m1 /m0 , and m2 /m0 (P < 0.001) as well as VO2 peak%, breathing reserve (BR), minute ventilation (VE)/VO2 (P < 0.001), and VE/carbon dioxide release (VCO2 ) (P = 0.006) correlated significantly with FEV1 %. LCI, m1 /m0 , and m2 /m0 correlated with VO2 peak (P ≤ 0.001), VE (L/min) (P < 0.05), BR (P < 0.01), VE/VO2 (P < 0.001), and VE/VCO2 (P < 0.01). Multiple regression analysis showed that LCI could predict BR% (P < 0.001, r2 :0.272) and VE/VO2 (P < 0.001, r2 : 0.207) while LCI and FRC could predict VO2 peak% P < 0.001, r2 : 0.216) and VE/VCO2 (P < 0.001, r2 : 0.226). CONCLUSION: Ventilation inhomogeneity as indicated by increased LCI is associated with less efficient ventilation during strenuous exercise and negatively impacts exercise capacity in CF.

Exercise responses are related to structural lung damage in CF pulmonary disease.

INTRODUCTION: Early detection of lung disease is a primary objective in monitoring patients with Cystic Fibrosis (CF); High-Resolution-Computed-Tomography (HRCT) assesses structural damage. Spirometry and cardiopulmonary exercise testing are used for functional evaluation of CF lung disease. AIM: To evaluate the deterioration of exercise testing parameters over a 2-year period compared to the change of spirometry and HRCT parameters among CF patients. METHODS: Twenty-eight CF patients were evaluated with HRCT, spirometry, and exercise testing; 15 had two assessments with an interval of 2 years. Correlation analyses between Bhalla score parameters and functional measures were performed. RESULTS: Twenty-eight patients with CF (mean age 14.9 years, mean forced expiratory volume in 1 sec [FEV1 ] 83.2%) were evaluated. FEV1 was not found to change significantly in the 2-year period (P = 0.612). Both mean Bhalla score and mean peak oxygen consumption (VO2 peak %) deteriorated significantly (P = 0.014 and P = 0.026, respectively). VO2 peak and respiratory equivalents for O2 and CO2 at peak exercise were found to be significant predictors of Bhalla score (r = -0.477, P = 0.010; r = 0.461, P = 0.018; r = 0.402; P = 0.042, respectively). Anaerobic threshold was associated with changes in Bhalla score over the following 2 years. CONCLUSIONS: Exercise testing is more sensitive than spirometry to detect structural changes in CF lungs. Pediatr Pulmonol. 2016; 51:914-920. © 2016 Wiley Periodicals, Inc.

Pancreatic Cystosis in Two Adolescents with Cystic Fibrosis.

We present pancreatic cystosis in two adolescents with cystic fibrosis, a 13-year-old girl and an 18-year-old boy. In pancreatic cystosis, which is a rare manifestation of CF, the pancreatic parenchyma is replaced with multiple cysts of different sizes. Pancreatic cystosis is mainly an imaging based diagnosis and frequent follow-up should be recommended.