Low-frequency oscillometry indices to assess ventilation inhomogeneity in CF patients.

Background: The utility of the forced oscillations technique (FOT) in cystic fibrosis (CF) remains uncertain. The aim of this study was to explore the ability of lower-frequency FOT indices, alone and after adjustment for the lung volume, to assess the extent of ventilation inhomogeneity in CF patients with varying disease severity.

Methods: Forty-five children, adolescents, and adults with CF (age 6.

Mannitol Challenge to Assess Therapy Response in Asthmatic Children: An Interventional Cohort Study.

Bronchial provocation tests, such as the mannitol challenge, can be performed to identify and quantify the severity of bronchial hyperresponsiveness in asthmatic patients. Studies of the mannitol challenge as a monitoring tool in asthmatic children are limited. Our primary aim was to compare the bronchial hyperresponsiveness to mannitol in treatment-naive asthmatic children between baseline and three months after receiving the indicated asthma prophylaxis.

Physical Activity and Quality of Life among Patients with Cystic Fibrosis.

Background: Physical activity (PA) improves exercise capacity, slows the decline in lung function, and enhances Quality of Life (QoL) in patients with cystic fibrosis (pwCF).

Objectives: The study aimed to evaluate PA and QoL among children with CF compared to healthy controls; the secondary aim was to assess the correlation between PA, QoL, and lung function (FEV1).

Methods: Forty-five children and adolescents with CF and 45 age-matched controls completed two self-administered validated questionnaires: The Godin Leisure-Time Exercise Questionnaire (GLTEQ) and the DISABKIDS for QoL.

Hellenic registry of patients with home mechanical ventilation (HR-HMV): profiling sleep Apnea-Hypopnea syndrome patients across Greece.

Background: Chronic respiratory conditions are a prominent public health issue and thus, building a patient registry might facilitate both policy decision making and improvement of clinical management processes. Hellenic Registry of patients with Home Mechanical Ventilation (HR-HMV) was initiated in 2017 and a web-based platform is used to support patient data collection. Eighteen hospital departments (including sleep labs) across Greece participate in this initiative, focusing on recording data for both children and adult patients supported by mechanical ventilation at home, including patients with Sleep Apnea-Hypopnea Syndrome (SAHS) under Positive Airway Pressure (PAP) therapy.

Pulmonary exacerbations, airway pathogens, and long-term course of lung clearance index in children and young adults with cystic fibrosis.

Background: Pulmonary exacerbations (PEx), pathogens colonizing the respiratory tract, and patients' age are associated with progressive worsening of lung function among patients with cystic fibrosis (CF). However, the effect of these factors on longitudinal changes of Lung Clearance Index (LCI) remains unclear.

Aim: To assess the role of age, different types of bronchial infection, and PEx on LCI deterioration.

Pulmonary medication adherence among children and adults with cystic fibrosis: Is there an association with disease severity?

Background: Adherence to pulmonary medication is pivotal in delaying the progression of lung disease in cystic fibrosis (CF). Further exploring the consequences of poor adherence and its impact on disease severity may be valuable to personalize CF treatment strategy.

Aim: To evaluate indicators of disease severity among children and adults with CF and investigate which of them are related to pulmonary medication adherence.

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.

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.

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.

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.

Cardiorespiratory Fitness Predicted by Fibrinogen and Leptin Concentrations in Children with Obesity and Risk for Diabetes: A Cross-Sectional Study and a ROC Curve Analysis.

Obesity is defined as abnormal or excessive fat accumulation that presents a risk to health. The ability to exercise is affected by adiposity, and this mechanism involves low-grade chronic inflammation and homeostatic stress produced mainly in adipocytes, which can result in abnormal adipokine secretion. To date, the gold standard for cardiorespiratory fitness assessment is considered to be the maximum oxygen uptake (VO).

Does 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 colonization status affects Exercise Capacity, LCI and High-Resolution Computed Tomography (HRCT) indices among patients with CF; to check if colonization can predict exercise intolerance.

Subjects: Seventy-eight (78) children and adults with CF (31 males) mean (range) age 17.

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/ΔVCO or V'E/V'CO slope) and intercept of ventilation (VE-intercept) in CF patients with mild, moderate, and severe cystic fibrosis (CF) lung disease.

Two cases of myositis ossificans in children, after prolonged immobilization.

Myossitis ossificans (MO) is a benign disorder characterized by heterotopic bone formation in skeletal muscle. It is divided in three types, fibrodysplasia ossificans progressive (FOP), myositis ossificans circumscripta or traumatica (MOT) and myositis ossificans without a history of trauma (non traumatic or pseudomalignant MO). Myositis ossificans is extremely rare in children younger than 10 years.

Chest wall lipoblastoma in a 3 year-old boy.

Background: Lipoblastoma is a rare, benign, fatty tissue tumor that occurs in infancy and early childhood. The most common tumor locations are the extremities and the torso. The location of this tumor in the chest wall and an intrathoracic extension is uncommon.

A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung Disease.

Novel targeted treatments for Cystic Fibrosis give rise to new hope for an ever-growing number of CF patients with various mutations. However, very little evidence and guidelines exist to steer clinical decisions regarding patients whose illness takes an unexpected course. In such cases, the benefits and risks of discontinuing these treatments must be carefully and individually weighed, since their long-term effects remain mainly uncharted territory.

Efficacy and safety of the combination fluticasone propionate plus salmeterol in asthmatic preschoolers: An observational study.

Background: Inhaled Corticosteroids (ICS) are the cornerstone of asthma management in pediatric patients. However, in some cases, asthma is not adequately controlled on ICS alone. Long-acting beta-agonists (LABA) are one of the available additional therapies but their use has rarely been studied among children younger than 5 years.

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

Introduction: FEV 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.

A "real-life" study on height in prepubertal asthmatic children receiving inhaled steroids.

Introduction: Asthma is the most common chronic respiratory disease in children and inhaled corticosteroids (ICS) constitute the first line of treatment for these patients. However, the potential growth-inhibiting effect of ICS has often been a cause of concern for both caregivers as well as physicians, and there still remains conflict regarding their safety profile.

Objective: To assess whether the administration of ICS in low or medium doses is associated with height reduction in prepubertal children.

Assessment of IgE-mediated food allergies in children with atopic dermatitis.

Background: Atopic dermatitis (AD) is an inflammatory disease of the skin, which is characterised by a chronic relapsing course.

Aim: The aim of the study was to assign the prevalence of clinically active food allergies among a group of children between 3 months and 7 years of age, with AD.

Methods: Eighty-eight children with AD were screened for specific IgE antibodies to food proteins.

Home intravenous antibiotic therapy in children with cystic fibrosis: clinical outcome, quality of life and economic benefit.

Background: Pediatric home care has improved therapeutic options for children with chronic disease. Home intravenous (IV) antibiotic treatment against (PsA) in cystic fibrosis (CF) patients has offered increased flexibility to these patients and family life. A prospective clinical study was conducted to compare safety, efficacy, and cost benefits of home versus hospital IV antibiotic treatment among CF children and adolescents.

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.

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.