Childhood interstitial lung disease survivors in adulthood: a European collaborative study.

Background: Interstitial lung disease (ILD) is rarer in children (chILD) than adults, but with increasing diagnostic awareness, more cases are being discovered. chILD prognosis is often poor, but increasing numbers are now surviving into adulthood.

Aim: To characterize chILD-survivors and identify their impact on adult-ILD centers.

Pulmonary manifestations of Pseudohypoaldosteronism type 1b: A systematic review of the literature.

Introduction: Pseudohypoaldosteronism type 1b (PHA1B) is a rare autosomal recessive disease caused by dysfunction of amiloride-sensitive epithelial sodium channels (ENaC), that might present with a wide variety of pulmonary symptoms.

Methods: We provide a systematic review of published cases with PHA1B and respiratory symptoms, adding a relevant case from our clinic.

Results: Thirty-seven publications presenting 61 cases were identified apart from our case.

An update on diagnosis and management of obstructive sleep apnoea in the first 2 years of life.

The aim of this review is to summarise evidence that became available after publication of the 2017 European Respiratory Society statement on the diagnosis and management of obstructive sleep apnoea syndrome (OSAS) in 1- to 23-month-old children. The definition of OSAS in the first 2 years of life should probably differ from that applied in children older than 2 years. An obstructive apnoea-hypopnoea index >5 events·h may be normal in neonates, as obstructive and central sleep apnoeas decline in frequency during infancy in otherwise healthy children and those with symptoms of upper airway obstruction.

Management of Persistent, Post-adenotonsillectomy Obstructive Sleep Apnea in Children: An Official American Thoracic Society Clinical Practice Guideline.

Obstructive sleep apnea (OSA) is the most common sleep-related breathing disorder. Although adenotonsillectomy is first-line management for pediatric OSA, up to 40% of children may have persistent OSA. This document provides an evidence-based clinical practice guideline on the management of children with persistent OSA.

Inflammation and Infection in Cystic Fibrosis: Update for the Clinician.

Inflammation and infection play an important role in the pathophysiology of cystic fibrosis, and they are significant causes of morbidity and mortality in CF. The presence of thick mucus in the CF airways predisposes to local hypoxia and promotes infection and inflammation. A vicious cycle of airway obstruction, inflammation, and infection is of critical importance for the progression of the disease, and new data elucidate the different factors that influence it.

Adenotonsillectomy: the good, the bad and the unknown.

Purpose Of Review: Adenotonsillar hypertrophy is the most common pathogenetic contributor to obstructive sleep apnea syndrome (OSAS) in childhood, and adenotonsillectomy is the standard initial treatment. Here, we summarize the most recent evidence on the efficacy and complications of adenotonsillectomy and explore knowledge gaps in clinical management.

Recent Findings: Favorable adenotonsillectomy effects have been reported in children with very severe OSAS [apnea-hypopnea index (AHI) >20 episodes/h] and extremely severe OSAS (AHI >100 episodes/h), without postoperative mortality, need for endotracheal intubation, prolonged hospital stay or re-admission after hospital discharge.

Changes in Ventilatory Support Requirements of Spinal Muscular Atrophy (SMA) Patients Post Gene-Based Therapies.

Spinal muscular atrophy (SMA) is a genetic neuromuscular disease resulting in global muscular weakness and, frequently, in respiratory failure and premature death. Gene-based therapies like Nusinersen are now available for patients with SMA. The aim of this review was to assess in "real world" studies, whether novel treatments would have a positive impact on the mechanical ventilatory support requirements of SMA patients, already initiated on ventilatory support prior to treatment administration.

Nocturnal oximetry parameters as predictors of sleep apnea severity in resource-limited settings.

Nocturnal oximetry is an alternative modality for evaluating obstructive sleep apnea syndrome (OSAS) severity when polysomnography is not available. The Oxygen Desaturation (≥3%) Index (ODI3) and McGill Oximetry Score (MOS) are used as predictors of moderate-to-severe OSAS (apnea-hypopnea index-AHI >5 episodes/h), an indication for adenotonsillectomy. We hypothesised that ODI3 is a better predictive parameter for AHI >5 episodes/h than the MOS.

Autoimmune pulmonary alveolar proteinosis in children.

In childhood, a multitude of causes lead to pulmonary alveolar proteinosis (PAP), an excessive surfactant accumulation in the alveolar space, limiting gas exchange. Autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) causing autoimmune PAP, the principal aetiology in adults, are rare. In this first case series on autoimmune PAP, we detail the presentation and management issues of four children.

Low- and High-Attenuation Lung Volume in Quantitative Chest CT in Children without Lung Disease.

In contrast to studies of adults with emphysema, application of fixed thresholds to determine low- and high-attenuation areas (air-trapping and parenchymal lung disease) in pediatric quantitative chest CT is problematic. We aimed to assess age effects on: (i) mean lung attenuation (full inspiration); and (ii) low and high attenuation thresholds (LAT and HAT) defined as mean attenuation and 1 SD below and above mean, respectively. Chest CTs from children aged 6-17 years without abnormalities were retrieved, and histograms of attenuation coefficients were analyzed.

Accuracy of the sleep clinical record for the diagnosis of pediatric moderate-to-severe obstructive sleep apnea syndrome.

Purpose: The sleep clinical record (SCR) has been used to diagnose obstructive sleep apnea syndrome (OSAS) in children when access to polysomnography (PSG) is limited. Our aim was to determine the best SCR score that could facilitate diagnosis of moderate-to-severe OSAS in children with snoring.

Methods: Healthy children with history of snoring, who were referred for PSG, were prospectively recruited.

Effects of the COVID-19 lockdown on sleep duration in children and adolescents: A survey across different continents.

Background: A parent survey was conducted to assess the sleep habits of children residing in various countries before and during the SARS-CoV-2 pandemic. It was hypothesized that lockdown would be associated with increased sleep duration.

Methods: Outcomes were changes in bedtime, wake time, and sleep duration in the pandemic compared to before.

Phenotypic variance in pediatric obstructive sleep apnea.

It is crucial that clinicians understand what underpins the considerable phenotypic variance in pediatric obstructive sleep apnea syndrome (OSAS), if they are to implement individually tailored phenotype-based approaches to diagnosis and management. This review summarizes the current literature on how disease severity, comorbidities, genetic and environmental/lifestyle factors interact to determine the overall OSAS phenotype. The first part discusses the impact of these factors on OSAS-related morbidity in the context of otherwise healthy children, whilst the second half details children with complex conditions, particularly focusing on the anatomical and functional abnormalities predisposing to upper airway obstruction unique to each condition.

Lung hyperinflation quantitated by chest CT in children with bronchiolitis obliterans syndrome following allogeneic hematopoietic cell transplantation.

Objectives: Bronchiolitis obliterans syndrome (BOS) diagnosis in children following allogeneic hematopoietic stem cell transplantation (post-HSCT) is based on detection of airway obstruction on spirometry and air-trapping, small airway thickening or bronchiectasis on chest CT. We assessed the relationship between spirometry indices and low-attenuation lung volume at total lung capacity (TLC) on CT.

Methods: Data of children post-HSCT with and without BOS were analyzed.

Triage for Malnutrition Risk among Pediatric and Adolescent Outpatients with Cystic Fibrosis, Using a Disease-Specific Tool.

Malnutrition prevails in considerable proportions of children with Cystic Fibrosis (CF), and is often associated with adverse outcomes. For this, routine screening for malnutrition is pivotal. In the present cross-sectional study, we aimed to assess the risk for malnutrition in pediatric outpatients with CF.

Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study.

Nutrition is an important component of cystic fibrosis (CF) therapy, with a high-fat diet being the cornerstone of treatment. However, adherence to the dietary recommendations for CF appears suboptimal and burdensome for most children and adolescents with CF, leading to malnutrition, inadequate growth, compromised lung function and increased risk for respiratory infections. A cross-sectional approach was deployed to examine the degree of adherence to the nutrition recommendations and diet quality among children with CF.

Nebulizer Care and Inhalation Technique in Children with Cystic Fibrosis.

Nebulizers are used by the great majority of cystic fibrosis patients for delivery of cornerstone treatments. Inhalation technique and adequate disinfection and maintenance are important for optimizing medication delivery. In this study, inhalation technique and nebulizer disinfection/maintenance were assessed in cystic fibrosis patients by direct observation in clinic and completion of a scoring sheet.

Measures of nocturnal oxyhemoglobin desaturation in children with neuromuscular disease or Prader-Willi syndrome.

Objectives: Evidence for nocturnal oximetry interpretation in patients with abnormal neuromuscular function is limited. We aimed to compare children with neuromuscular disease (NMD) or Prader-Willi syndrome (PWS) to otherwise healthy subjects with obstructive sleep-disordered breathing (SDB) or without respiratory disorder (controls) regarding nocturnal oximetry parameters.

Methods: We analyzed recordings from children with: (a) NMD; (b) PWS; (c) snoring and adenotonsillar hypertrophy and/or obesity (SDB); and (d) controls.

Frequency of moderate-to-severe obstructive sleep apnea syndrome among children with snoring and blood pressure in the hypertensive range.

Background: It is recommended that children with hypertension and loud snoring should be referred for polysomnography. We aimed to compare the frequency of moderate-to-severe obstructive sleep apnea syndrome (OSAS) among snorers with and without hypertension. Thus, it was hypothesized that systolic or diastolic hypertension among children with snoring is a risk factor for moderate-to-severe OSAS.