The real-life experience of a general pulmonary transition clinic.

Background: Paediatric-to-adult transition represents an unmet need in many chronic conditions. Data and -outcomes on pulmonary transition clinics (PTC) are limited.  Methods: We report the structure, organization, and patients' characteristics of a multidisciplinary outpatient PTC started in 2022 by two secondary level academic hospitals in Milan, Italy.

Congenital Bronchial Atresia: To Surgically Treat or Conservatively Manage? A Systematic Review.

Background: No standardized guidelines exist for the management of isolated congenital bronchial atresia (CBA), particularly in asymptomatic cases. This systematic review analyzes both pediatric and adult cases to inform future treatment recommendations.

Methods: A systematic review was conducted following PRISMA guidelines, including studies of pediatric (<15 years) and adult (≥15 years) patients with CBA.

Lung function trajectories in children with early diagnosis of non-cystic fibrosis bronchiectasis: a retrospective observational study.

Background: Non-cystic fibrosis (non-CF) bronchiectasis (BE) is defined as a clinical syndrome of recurrent, persistent wet cough and abnormal bronchial dilatation on chest High Resolution Computed Tomography (HRCT) scans. The aims of this study were to characterize the pattern of the trajectories of lung function parameters and to consider the relationship between the lung function and radiological severity according to the modified Reiff score.

Methods: The study retrospectively considered 86 children (46.

Lung Diseases and Rare Disorders: Is It a Lysosomal Storage Disease? Differential Diagnosis, Pathogenetic Mechanisms and Management.

Pulmonologists may be involved in managing pulmonary diseases in children with complex clinical pictures without a diagnosis. Moreover, they are routinely involved in the multidisciplinary care of children with rare diseases, at baseline and during follow-up, for lung function monitoring. Lysosomal storage diseases (LSDs) are a group of genetic diseases characterised by a specific lysosomal enzyme deficiency.

Lung Function Evaluated By Structured Light Plethysmography in Children After Lung Surgery: A Preliminary Analysis.

Structured light plethysmography (SLP) is a novel light-based method that captures chest wall movements to evaluate tidal breathing. Thirty-two children who underwent lung surgery were enrolled. Their clinical history was collected along with spirometry and SLP.

Recurrent Respiratory Infections in Children with Down Syndrome: A Review.

Down Syndrome (DS) is the most common chromosomal abnormality compatible with life. The life of patients suffering from DS can be strongly impacted by Recurrent Respiratory tract Infections (RRIs), leading to an increased rate of hospitalisation, a higher need for intensive care and fatality. With a literature review, we summarise here the main etiological factors for RRI in this category of patients, particularly focusing on airway malformations such as tracheomalacia, tracheal bronchus and bronchomalacia, comorbidities associated with the syndrome, like congenital heart diseases, dysphagia, gastroesophageal reflux, musculoskeletal involvement and obesity, and immunologic impairments, involving both innate and adaptive immunity.

Perimenstrual Asthma and Premenstrual Disorders in Adolescents with Asthma.

Background: Asthma is a common chronic disease in pediatric patients, and perimenstrual asthma (PMA), refers to the worsening of asthma symptoms during the perimenstrual period, mainly reported in adult women. However, there is limited information regarding the exacerbation of symptoms in the presence of premenstrual disorders (PMDs) in adolescents. The aim of this pilot observational study was to investigate the frequency and potential association of PMA and PMDs in a clinical sample of adolescents with asthma.

Invasive Group A streptococcal infections: are we facing a new outbreak? A case series with the experience of a single tertiary center.

Background: In pediatric age, Group A Streptococcus (GAS) is responsible for a wide spectrum of clinical manifestations, from mild localized infections to life-threatening invasive diseases. In December 2022, the World Health Organization reported an increased incidence of scarlet fever and invasive GAS infections (iGAS) cases in Europe and the United States. In line with these observations, surveillance has been strengthened in our Region, allowing the identification of certified or highly suspected forms of iGAS.

Lung Function in Childhood: Designing the Future Trajectories.

Lung development is a highly regulated process that may be disrupted by both genetic and environmental factors beginning at an early age [...

Pediatric obesity and severe asthma: Targeting pathways driving inflammation.

Asthma affects more than 300 million people of all ages worldwide, including about 10-15% of school-aged children, and its prevalence is increasing. Severe asthma (SA) is a particular and rare phenotype requiring treatment with high-dose inhaled corticosteroids plus a second controller and/or systemic glucocorticoid courses to achieve symptom control or remaining "uncontrolled" despite this therapy. In SA, other diagnoses have been excluded, and potential exacerbating factors have been addressed.

Hypertensive pneumothorax with cystic lesions: Pleuropulmonary blastoma in an infant.

Pleuropulmonary blastoma (PPB) is a rare primitive malignant lung cancer that occurs in pediatric age. Its main differential diagnosis is congenital cystic pulmonary malformation (CPAM). A 30-day-old infant with respiratory failure obtained a chest x-ray and a computed tomography scan (CT) which revealed hypertensive pneumothorax with multifocal bilateral cysts.

Congenital Lung Malformations: Clinical and Functional Respiratory Outcomes after Surgery.

Congenital lung malformations (CLMs) involve anomalies of the lungs and respiratory tree such as congenital pulmonary airway malformation (CPAM), pulmonary sequestration (PS), bronchogenic cysts, congenital lobar emphysema, and bronchial atresia (BA). Although symptomatic lesions require surgical resection, the appropriateness of surgery for patients with asymptomatic malformations is a matter of ongoing debate. Limited data are available concerning the long-term follow-up of affected subjects.

Lung involvement in children with COVID-19 multisystem inflammatory syndrome.

Since the beginning of the COVID-19 pandemic, multisystem inflammatory syndrome in children (MIS-C) has been reported in increasing numbers, mostly focusing on cardiac dysfunction. Very few studies have evaluated lung involvement in terms of imaging findings, while data regarding pulmonary function in children with MIS-C are not available. The purpose of our study was to evaluate lung involvement in MIS-C by imaging and lung function by structured light plethysmography (SLP) at hospital admission and 6 months afterwards.

The impact of adiposity indices on lung function in children with respiratory allergic diseases.

Introduction: The effect of obesity on lung function in children stratified by asthma status is not fully elucidated. We evaluated the impact of adiposity indices, including Body Mass Index (BMI) and estimated fat mass (eFT), on lung changes in asthmatic and non-asthmatic children with rhinitis.

Patients And Methods: We performed a retrospective review of 400 pediatric patients, classified into an asthma group (n = 200) and a no-asthma group (n = 200).

Immunonutrition and SARS-CoV-2 Infection in Children with Obesity.

Since the beginning of the SARS-CoV-2 pandemic, there has been much discussion about the role of diet and antiviral immunity in the context of SARS-CoV-2 infection. Intake levels of vitamins D, C, B12, and iron have been demonstrated to be correlated with lower COVID-19 incidence and mortality. Obesity has been demonstrated to be an independent risk for the severity of COVID-19 infection in adults and also in children.

Long COVID-19 in Children: From the Pathogenesis to the Biologically Plausible Roots of the Syndrome.

Long Coronavirus disease-19 (COVID-19) refers to the persistence of symptoms related to the infection with severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2). This condition is described as persistent and can manifest in various combinations of signs and symptoms, such as fatigue, headache, dyspnea, depression, cognitive impairment, and altered perception of smells and tastes. Long COVID-19 may be due to long-term damage to different organs-such as lung, brain, kidney, and heart-caused by persisting viral-induced inflammation, immune dysregulation, autoimmunity, diffuse endothelial damage, and micro thrombosis.

Barrier Impairment and Type 2 Inflammation in Allergic Diseases: The Pediatric Perspective.

Allergic diseases represent a global burden. Although the patho-physiological mechanisms are still poorly understood, epithelial barrier dysfunction and Th2 inflammatory response play a pivotal role. Barrier dysfunction, characterized by a loss of differentiation, reduced junctional integrity, and altered innate defence, underpins the pathogenesis of allergic diseases.

Pediatric Obesity-Related Asthma: The Role of Nutrition and Nutrients in Prevention and Treatment.

Childhood obesity rates have dramatically risen in numerous countries worldwide. Obesity is likely a factor in increased asthma risk, which is already one of the most widespread chronic respiratory pathologies. The pathogenic mechanism of asthma risk has still not yet been fully elucidated.

Airway Malacia: Clinical Features and Surgical Related Issues, a Ten-Year Experience from a Tertiary Pediatric Hospital.

Background: Few studies have been carried out with the aim of describing the clinical course and follow-up of patients with tracheomalacia. We aim to describe the symptoms at diagnosis and the post-treatment clinical course of patients affected by airway malacia.

Methods: We retrospectively analyzed characteristics of pediatric patients with a diagnosis of airway malacia.

The Diabetic Lung: Insights into Pulmonary Changes in Children and Adolescents with Type 1 Diabetes.

Historically, the lung was not listed and recognized as a major target organ of diabetic injury. The first evidence of diabetic lung involvement was published fifty years ago, with a study conducted in a population of young adults affected by type 1 diabetes (T1D). In recent years, there has been mounting evidence showing that the lung is a target organ of diabetic injury since the beginning of the disease-at the pediatric age.

Relationship between quality of life and behavioural disorders in children with persistent asthma: a Multiple Indicators Multiple Causes (MIMIC) model.

Knowledge on multiple interdependences between quality of life (QoL) and behavioural problems in relation to asthma severity and control is undetermined. The aims of the study were: (i) to assess the relationship of QoL and behavioural problems with asthma severity and control (ii) to predict children's "abnormal/borderline" status with variation in QoL. For these purposes a multicenter case-control study on 47 Severe Asthma (SA) and 94 Moderate Asthma (MA) children was performed.

Airway inflammation and injury in children with prevalent weakly acidic gastroesophageal refluxes.

Background: In children with gastroesophageal reflux (GER) both acid refluxes (AR) and weakly acidic refluxes (WAR) can induce respiratory symptoms (RS).

Methods: To characterize the airway inflammation in children with more prevalent WAR or AR (defined according a ROC curve analysis), we performed a 3 year-retrospective review of the medical records of patients who underwent fiberoptic bronchoscopy for difficult-to-treat chronic/recurrent respiratory symptoms and who had a positive multiple intraluminal esophageal impedance (pH/MII) monitoring.

Results: In the 13 WAR and 11 AR children, the number of cells recovered by bronchoalveolar lavage (BAL) was similar [0.

Feasibility of structured light plethysmography for the evaluation of lung function in preschool children with asthma.

Background: Structured light plethysmography (SLP) is a new noninvasive technology to capture the movement of the thoracic and abdominal wall, and to assess some parameters indicative for lung function.

Objective: The purpose of the study was to evaluate the feasibility of SLP in children with asthma.

Methods: A total of 52 patients were enrolled: 25 with asthma exacerbation (group 1), 13 with well-controlled asthma (group 2), and 14 healthy controls (group 3).