Background: Severe asthma represents a significant challenge for children and adolescents. At the same time, it often places a burden on patients, caregivers, and society, mainly related to morbidity, mortality, and health care resources. In children and adolescents, severe asthma is mostly characterized by type 2 inflammation, which leads to bronchial eosinophilia that may be suppressed by corticosteroids

Background: Autosomal recessive cutis laxa type IC (ARCL1C) is characterized by cutis laxa accompanied by pulmonary, gastrointestinal, urinary, musculoskeletal involvement caused by biallelic mutations in latent transforming growth factor-beta binding protein 4 (LTBP4) gene. The overall prognosis is poor, and most patients die in infancy because of severe pulmonary emphysema (PE).

Background: Peanut introduction guidelines have undergone significant reversal since 2001 from recommending delayed introduction to rescinding the recommendations in 2008 to actively recommending early introduction of peanut between 4 and 11 months of age in high-risk infants in 2015. This qualitative study aims to explore pediatrician beliefs, practices, facilitators, and barriers regarding peanut

Reduced exercise tolerance in a patient in whom any heart diseases were preliminarily excluded suggests other frequent causes of effort-related symptoms, that is, exercise-induced bronchoconstriction, lack of training, or overweight. The presented case underscores the importance of comprehensive diagnostic workup in children with exercise-related symptoms.

Bronchopulmonary dysplasia (BPD) has evolved considerably since its first description over 50 years ago. This review aims to provide a historical framework for conceptualizing BPD and a current understanding of the changing definition, epidemiology, pathophysiology, treatment, and outcomes of BPD. The transdisciplinary approach that led to the initial phenotypic description of BPD continues to hold

Importance: There is increasing literature on the role of health literacy in asthma outcomes. Despite less than half of Americans having proficient health literacy, health literacy is an essential component in providing quality medical care.

Background: Hereditary angioedema (HAE) due to C1 inhibitor (C1INH) deficiency is characterized by recurrent attacks of edema of the skin and mucosal tissues. Symptoms usually present during childhood (mean age at first attack, 10 years). Earlier symptom onset may predict a more severe disease course. Subcutaneous (SC) C1INH is indicated for routine prophylaxis to prevent HAE attacks in adolescents

Background: Childhood asthma carries significant morbidity.

Introduction: Pulmonary aspiration in infants and children is common, with 25% of the pediatric population reported to experience some type of nonspecific swallowing dysfunction. Diagnosing and managing oropharyngeal aspiration remain a challenging task due to the lack of distinguishing clinical or laboratory characteristics. We conducted a large, physician-based survey in an effort to determine the

Background: The parameters of lung sounds have been suggested as biomarkers of airway changes. Using a commercially available lung sound analyzer, we investigated the characteristics of the lung sounds in infants with acute respiratory infection (ARI).

Background: Biologics are the cornerstone of therapy in children and adolescents with severe or uncontrolled allergic diseases, such as asthma, atopic dermatitis, and chronic urticaria. Since the World Health Organization (WHO) declared severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection a pandemic in March 2020, some scientific societies have released statements on the use of biologics

Background: Recombinant factor VIIa (rFVIIa) is a highly purified recombinant protein. It is approved for the treatment and prevention of bleeding episodes associated with congenital factor VII deficiency, congenital hemophilia with inhibitors, and Glanzmann's thrombasthenia. The most commonly reported adverse events are thrombolytic in nature. In this report, we present a successful desensitization

Introduction: Echinocandin antifungal medications including micafungin are being used more commonly in the treatment of invasive fungal infections in both pediatric and adult patients. Micafungin is also a first-line therapeutic option for candidemia and antifungal prophylaxis in a variety of clinical settings. Hypersensitivity reactions have not been well described; however, isolated cases have been

Severe Acute Respiratory Syndrome coronavirus 2 (SARS-CoV-2), the etiologic agent of the disease COVID-19, first emerged in late December 2019 in China, and has subsequently become a pandemic with unprecedented clinical impact. The virus appears to more severely affect older individuals and those with co-morbid medical conditions, specifically those with chronic lung disease, obesity, heart failure

As the coronavirus disease 2019 (COVID-19) pandemic continues globally, there are an increasing number of pediatric cases. Since the initial symptoms of COVID-19 may overlap and co-exist with other respiratory infections typically affecting children, this review examines the use of computed tomography as a diagnostic and management tool. Children with preexisting conditions may be particularly prone

Background: Acute bronchiolitis is one of the most common diseases of early childhood. There are many recent changes in the treatment of acute bronchiolitis. The aim of this study is to evaluate treatment approaches to acute bronchiolitis among clinicians and to observe compliance with clinical guidelines.

Background: Clinical trials of 7% hypertonic saline (HTS) in cystic fibrosis (CF) show short- and long-term benefits, including improved pulmonary function and reduced exacerbation risk. Adverse effects of HTS include bronchospasm, and guidelines recommend tolerance be tested in a clinical environment before prescribing. We evaluated the rate of, and risk factors for, intolerance of HTS at a single

Background: Atopic dermatitis (AD) is a common inflammatory skin disease with complex pathogenesis. Natural flavonoids exhibit strong anti-inflammatory and antioxidant properties in many human diseases. In this study, the potential bioactive effect of quercetin, a polyphenolic plant-derived flavonoid, on the AD model of human keratinocytes was evaluated.

Background: Thiols and ischemia-modified albumin (IMA) are novel biomarkers for the evaluation of the oxidative stress (OS) that has been associated with the pathogenesis of various neoplastic and inflammatory diseases. The aim of this study was to find out the dynamic thiol–disulfide homeostasis and IMA levels in patients with cutaneous mastocytosis (CM) to demonstrate the presence of the OS.

Background: Asthma continues to be the leading chronic disease affecting children in the United States. With mounting evidence of how diet plays a role in both chronic and allergic diseases, it is important to identify types of foods that may potentially promote a pro-inflammatory state. The study aims to examine the association between current asthma prevalence and intake of specific foods among children

Introduction: In recent years, administration of inhaled aminoglycosides has gained popularity in tracheostomy-dependent pediatric patients because of medication delivery to the target site of action while minimizing systemic absorption and adverse effects. A recent report of detectable serum tobramycin concentrations in critically ill children receiving inhaled tobramycin 300 mg every 12 h prompted

The data on perioperative anaphylaxis (PA) in children is limited and usually reported with neuromuscular blocking agents and antibiotics. However we present a first pediatric case who developed PA with paracetamol unlike the literature.

Upper respiratory tract infection (URTI)-associated acute cough is the most common symptom both in children and adults worldwide and causes economic and social problems with significant implications for the patient, the patient's family, and the health care system. New pathogenic mechanisms in acute cough, including the urge to cough (UTC) mechanisms, have been recently identified. The brainstem neural

The explanation of hypersensitivity reactions has long relied on the classification of Gell and Coombs, originally proposed in the 1960s. However, their concepts were predated by other authors by at least 50 years. A 21st century pediatric allergy clinic provides multiple examples of these basic concepts on a daily basis. We review classic and less classic examples of the original criteria, highlight

Background: Primary immunodeficiencies (PIDs) are a heterogeneous group of congenital disorders characterized by susceptibility to recurrent infections, allergy, malignancies and autoimmunity. The identification of disease-causing genetic defects is critically important for treatment options. In last decade, next-generation sequencing (NGS)-based methods has enabled the rapid genetic screening and

Background: T helper 17 (Th17) is regarded as key immune cell in the pathogenesis of noneosinophilic asthma (NEA) due to the recruitment of neutrophils into the airways. The mammalian target of rapamycin (mTOR) is an important signaling molecule that plays a critical role in immune regulation. This study focused on mTOR signaling pathway in the regulation of Th17-mediated neutrophilic airway inflammation

Background: Up to 40% of the world populations are affected by allergic rhinitis (AR). Interplay between genetics, epigenetics, and environmental factors leads to allergic disease.

Asthma guidelines provide clinicians with evidence-based management strategies for this chronic condition. The preferred therapy for patient with persistent asthma is inhaled corticosteroids. However, ∼40% of the patients with persistent asthma continue to present with symptoms while treated according to the guidelines. Multiple factors are being explored to explain the variability in response to inhaled

Background: Stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) was first described in 2014 as a type I interferonopathy resulting from heterozygous mutations in the transmembrane protein 173 (TMEM173) gene. SAVI is characterized by the neonatal onset of systemic inflammation, severe cutaneous vasculopathy, and interstitial lung disease. Janus kinase inhibitors

Glomus tumors (GTs) are rare, usually benign, mesenchymal neoplasms typically located in the cutaneous tissues of the extremities. Visceral locations have been reported in ∼5% of cases. The average age at diagnosis is 42 years. GTs originating in the respiratory tract of pediatric patients are exceedingly rare. We report a 16-year-old male with a GT of the right lower lobe bronchus.

Introduction: Pediatric noncystic fibrosis (CF) bronchiectasis has a variety of causes. An early and accurate diagnosis may prevent disease progression and complications. Current diagnostics and yield regarding etiology are evaluated in a pediatric cohort at a tertiary referral center. Methods: Available data, including high-resolution computed tomography (HRCT) characteristics, microbiological testing

Background: Pulmonary exacerbations (PExs) are common in individuals with cystic fibrosis (CF). Data regarding outcomes of outpatient parenteral antimicrobial therapy (OPAT) in children are sparse. Methods: Retrospective data of PEx episodes treated in the hospital versus OPAT collected. Children ≤18 years were included. Outcome measures included FEV1, FVC, FEF25-75%P, time to the next PEx, and weight

[This corrects the article DOI: 10.1089/ped.2016.0649.].

Rationale: Asthma is a common comorbid condition in sickle cell disease (SCD). However, obstructive lung disease is prevalent in SCD, independent of a diagnosis of asthma. It is speculated that the heightened state of inflammation in SCD, involving pathways distinct from allergic asthma, may underlie the SCD-specific obstructive disease. Objective: The objective of the study was to compare airway and

The National Asthma Education and Prevention Program Guidelines for the Diagnosis and Management of Asthma will update the guidelines on 6 topic areas. This review summarizes the findings for the charge to assess effectiveness of intermittent inhaled corticosteroid therapy with and without long-acting beta-agonist therapy in children 0 to 4 years of age, and children and adults 5 years and older. The

Impacted pediatric tracheobronchial thrombus is an uncommon diagnosis. There are no clearly proven therapeutic options for airway casts due to a similar process, plastic bronchitis. Cryotherapy, specifically cryoextraction, has shown potential as a therapeutic option in adults with tracheobronchial thrombus and cast. We describe the novel application of this method in a complex pediatric patient.

Background: In the medical complexity of a children with cerebral palsy, impaired airway clearance represents a major problem, leading to significative respiratory morbidity and mortality. Its management is difficult because of limited cooperation and poor tolerance to invasive treatments. Free Aspire® (FA) is a device designed to remove bronchial secretions noninvasively, without generating cough

Objective: To evaluate the early stage effects of adenoidectomy with/without tonsillectomy on immune functions in children aged <3 years. Methods: Twenty-four children aged <3 years underwent adenoidectomy with/without tonsillectomy were included. The levels of IgG, IgA, IgM, C3, and C4 were measured for humoral immunity, and the levels of CD3+, CD4+, CD8+, CD19+, CD56+, CD3+CD4-CD8-, and CD3+CD4+CD8+

Background: Bronchopulmonary dysplasia (BPD) is an important clinical problem for premature infants. Previously, some biomarkers associated with severity of BPD have been studied. In our study, we aimed to investigate the value of interleukin-33 (IL-33) levels as a new biomarker in the follow-up of BPD severity and response to treatment. Materials and Methods: Premature infants of <32 weeks of gestational

Pediatric acute respiratory distress syndrome (PARDS) is a significant cause of morbidity and mortality in children. Children with PARDS often require intensive care admission and mechanical ventilation. Unfortunately, beyond lung protective ventilation, there are limited data to support our management strategies in PARDS. The Pediatric Acute Lung Injury Consensus Conference (PALICC) offered a new

Background: Type 3 von Willebrand disease (VWD) is a severe bleeding disorder with a prevalence of 1:1 million live births. There are several von Willebrand factor (VWF) replacement products used in the treatment of acute bleeding episodes or as prophylaxis. Patients who receive multiple transfusions have an increased risk of developing antibodies against these products. These antibodies can lead to

Phenotypic variation in asthma, especially early childhood asthma, is increasingly recognized. Although inhaled corticosteroids are recommended as first-line therapy, it has less efficacy in controlling intermittent wheeze due to viral-induced symptoms in early childhood. This article reviews 2 emerging therapies in particular for early childhood wheeze: azithromycin and bacterial lysate therapy. Azithromycin's

Background: Subcutaneous immunoglobulin G (SCIG) may be a better option than intravenous immunoglobulin G (IVIG) for patients with primary immunodeficiencies (PID) due to reduced systemic and serious adverse reactions and easier administration. The Infusione Bimensile di Immunoglobuline Sottocute (IBIS) study investigated the effects of Hizentra®, a 20%-concentrated SCIG, administered biweekly in patients

Background: The association between vitamin D receptor (VDR) polymorphisms and the risk of asthma remains unclear. This study aimed to investigate the effect of VDR gene polymorphisms and VDR mRNA expression levels on respiratory function, nitric oxide levels in expiratory air, and serum vitamin D levels in children with asthma. Materials and Methods: The study included 80 healthy children (control

Background: Systemic corticosteroids are the standard of care for acute asthma exacerbation. Respiratory infections are known as common triggers of asthma exacerbation, but the risk of immune suppression from frequent periodic use of systemic steroids in poorly controlled asthmatic children is not well studied. Materials and Methods: We conducted a retrospective chart review of 26 children, 3-15 years

Background: Foreign body aspiration (FBA) is a serious life-threatening condition in childhood. "Baby-led weaning (BLW)" is a popular method in which the babies are encouraged to self-feed to gain oral motor abilities. The role of BLW in FBA is controversial. A retrospective study was performed to evaluate the results of FBA in infants (<1 year of age) and its relation to the feeding method. Materials

Background: There have been varying reports on the prevalence of antineutrophil cytoplasmic antibodies with bactericidal/permeability-increasing protein (BPI-ANCA) specificity in cystic fibrosis (CF) patients. These autoantibodies are believed to develop in response to infection and colonization, especially with Pseudomonas aeruginosa. The aim of this review was to estimate the overall prevalence of

Background: Severe postinfectious diffuse pulmonary disease may clinically mimic other entities of children's interstitial lung disease and is clinically challenging comprising various disease severities despite treatment. Long-term lung function trend and physical capacity in children with postinfectious diffuse pulmonary disease are rarely reported. We investigated trends in pulmonary function by

The respiratory tract can be colonized with bacterial, fungal, and viral microorganisms, and the whole of the microbiota, their genes, and the surrounding environment is collectively termed the microbiome. Increasing evidence indicates that the respiratory microbiome has an important role in respiratory health and disease and is both impacted by and potentially contributes to the severity of symptomatic

Background: Poor adherence with inhaled corticosteroid (ICS) medication is common in the pediatric population and can result in poor asthma control with increased frequency of asthma-related complications. The purpose of this study was to determine whether or not the initiation of ICS administration twice per day at school/daycare in patients with poor medication adherence at home improves asthma health

Background: The prevalence and impact of allergic and immune-mediated food disorders in pediatric acute-onset neuropsychiatric syndrome (PANS) are mostly unknown. Objective: We sought to explore the prevalence of atopic dermatitis (AD), asthma, allergic rhinitis (AR), IgE-mediated food allergies (FAs), and other immune-mediated food disorders requiring food avoidance in patients with PANS. In addition

Background: Under-perception of pulmonary dysfunction may delay appropriate treatment, while over-perception may result in unnecessary treatments. Objectives: To evaluate the ability of patients with asthma or cystic fibrosis and their subspecialty caregivers to assess changes in lung function based on their subjective clinical impressions. Methods: Patients were asked to qualitatively describe how

There is increasing recognition of phenotypic variability in pediatric asthma, providing the opportunity for a more personalized approach to therapy. Increasingly biologic therapies, in particular those targeting the "allergic" (or T helper 2) pathway, are being considered for children with severe asthma. However, there is a great deal of variability in the extent these biologic therapies have been

Background: Flexible bronchoscopy (FB) can be performed under bronchoscopist administered moderate sedation (BAMS) with a midazolam/fentanyl combination or general anesthesia (GA). However, the outcome of BAMS has not been well established in children. Currently, most of the centers prefer FB under GA. Both techniques have their advantages and disadvantages with implications for safety, complications

Childhood asthma represents a heterogeneous challenging disease, in particular in its severe forms. The identification of different asthma phenotypes has stimulated research in underlying molecular mechanisms, such as the endotypes, and paved the way to the search for related specific biomarkers, which may guide diagnosis, management, and predict response to treatment. A limited number of biomarkers

The rising prevalence of food allergy and specifically peanut allergy has had significant implications for affected patients, families, and society. The current standard of care remains strict avoidance and the use of emergency medications for accidental ingestions. There is recent evidence-based information to suggest that one approach to preventing peanut allergy lies in early introduction of peanut

An 11-month-old healthy infant girl was noted to have left lower lobe (LLL) opacities on chest X-ray (CXR) after developing desaturations during an elective cochlear implant surgery. Repeat CXR 10 days later revealed hyperexpansion of the left lung and persistent LLL opacity. Chest computerized tomography revealed enlarged mediastinal lymph nodes, left mainstem bronchial obstruction, and nodular opacities