Treatment of Nicotine Use in Adolescents Under 18 Years of Age: An Official American Thoracic Society Clinical Practice Guideline.

The rising popularity of electronic cigarettes (e-cigarettes), the nicotine product that is most used by adolescents since 2014, has reversed decades of progress in declining youth tobacco use. E-cigarette use in adolescents is associated with future smoking, and evidence is mounting of an increased association with nicotine dependence. Therapies used to treat nicotine dependence in adults include pharmacotherapy and behavioral interventions.

Genetic Testing Utilization in the U.S. Registry for Childhood Interstitial and Diffuse Lung Diseases.

Introduction: Childhood interstitial and diffuse lung diseases (chILD) comprise a diverse group of rare disorders. Identifying the underlying cause is crucial for treatment, prognosis, and estimating recurrence risk. The objective of this study was to assess the utilization of genetic testing for subjects enrolled in the United States National Registry for ChILD, a multicenter observational study.

Clinical Presentation and Diagnostic Evaluation of Vaping Related Lung Injury in Youth.

Background And Objectives: The vaping epidemic is a public health crisis worldwide. E-cigarette, or vaping product, use-associated lung injury (EVALI) was recognised in the summer of 2019 and resulted in more than 2800 hospitalizations and 60 deaths per the Centres for Disease Control and Prevention (CDC). Vaping refers to the use of E-cigarettes, which are electronic nicotine delivery systems (ENDS) and mimic smoking without combustion.

A progranulin variant causing childhood interstitial lung disease responsive to anti-TNF-α biologic therapy.

Background: Childhood interstitial and diffuse lung diseases are a collection of rare disorders with significant associated morbidity. Only a small subset of these diseases have precise diagnostic or therapeutic options identified to date.

Methods: Whole-exome sequencing in a family identified a candidate pathogenic variant predicted to be causing fibrotic lung and liver disease in a child.

Diagnosis of Post-Hematopoietic Stem Cell Transplantation Bronchiolitis Obliterans Syndrome in Children: Time for a Rethink?

Hematopoietic stem cell transplantation (HSCT) is undertaken in children with the aim of curing a range of malignant and nonmalignant conditions. Unfortunately, pulmonary complications, especially bronchiolitis obliterans syndrome (BOS), are significant sources of morbidity and mortality post-HSCT. Currently, criteria developed by a National Institutes of Health (NIH) working group are used to diagnose BOS in children post-HSCT.

Detection of Bronchiolitis Obliterans Syndrome after Pediatric Hematopoietic Stem Cell Transplantation: An Official American Thoracic Society Clinical Practice Guideline.

Many children undergo allogeneic hematopoietic stem cell transplantation (HSCT) for the treatment of malignant and nonmalignant conditions. Unfortunately, pulmonary complications occur frequently post-HSCT, with bronchiolitis obliterans syndrome (BOS) being the most common noninfectious pulmonary complication. Current international guidelines contain conflicting recommendations regarding post-HSCT surveillance for BOS, and a recent NIH workshop highlighted the need for a standardized approach to post-HSCT monitoring.

Innovations in the care of childhood interstitial lung disease associated with connective tissue disease and immune-mediated disorders.

Childhood interstitial lung disease (chILD) associated with connective tissue and immune mediated disorders is the second most common chILD diagnostic category. As knowledge of the molecular and genetic underpinnings of these rare disorders advances, the recognized clinical spectrum of associated pulmonary manifestations continues to expand. Pulmonary complications of these diseases, including ILD, confer increased risk for morbidity and mortality and contribute to increased complexity for providers tasked with managing the multiple organ systems that can be impacted in these systemic disorders.

Virtual interviews and equity: The pediatric pulmonary fellow perspective.

Background And Objectives: The SARS-CoV-2 pandemic shifted medical training programs to utilize virtual interviews (VIs) starting with the 2020 interview cycle. Fellowship interviews continue in the virtual format. It is unknown how this shift has affected equity for applicants as compared to in-person interviews.

Development of a Screening Algorithm for Lung Disease in Systemic Juvenile Idiopathic Arthritis.

Objective: Lung disease (LD) is an increasingly recognized complication of systemic juvenile idiopathic arthritis (sJIA). As there are no currently available guidelines for pulmonary screening in sJIA, we sought to develop such an algorithm at our institution.

Methods: A multidisciplinary workgroup was convened, including members representing rheumatology, pulmonary, stem cell transplantation, and patient families.

The US national registry for childhood interstitial and diffuse lung disease: Report of study design and initial enrollment cohort.

Introduction: Childhood interstitial and diffuse lung disease (chILD) encompasses a broad spectrum of rare disorders. The Children's Interstitial and Diffuse Lung Disease Research Network (chILDRN) established a prospective registry to advance knowledge regarding etiology, phenotype, natural history, and management of these disorders.

Methods: This longitudinal, observational, multicenter registry utilizes single-IRB reliance agreements, with participation from 25 chILDRN centers across the U.

Incidence and Risk Factors for Eosinophilia and Lung Disease in Biologic-Exposed Children With Systemic Juvenile Idiopathic Arthritis.

Objective: Although interleukin-1 (IL-1)/IL-6 inhibitors are effective therapies for systemic juvenile idiopathic arthritis (JIA), some patients develop eosinophilia and lung disease during treatment. This study was undertaken to retrospectively evaluate incidence and risk factors for eosinophilia and describe lung disease outcomes in IL-1/IL-6 inhibitor-exposed patients with systemic JIA.

Methods: Among JIA patients at our institution exposed to interleukin-1 (IL-1)/IL-6 inhibitors (1995-2022), we compared incidence rate of eosinophilia in systemic JIA compared to other JIA, stratified by medication class (IL-1/IL-6 inhibitors, other cytokine inhibitors, methotrexate).

An intronic variant in TBX4 in a single family with variable and severe pulmonary manifestations.

A male infant presented at term with neonatal respiratory failure and pulmonary hypertension. His respiratory symptoms improved initially, but he exhibited a biphasic clinical course, re-presenting at 15 months of age with tachypnea, interstitial lung disease, and progressive pulmonary hypertension. We identified an intronic TBX4 gene variant in close proximity to the canonical donor splice site of exon 3 (hg 19; chr17:59543302; c.

Pediatric EVALI in the Age of COVID-19/MIS-C: Diagnostic Considerations.

Objectives: Multisystem inflammatory syndrome in children (MIS-C) and e-cigarette or vaping product use-associated lung injury (EVALI) have significant overlap in clinical features, which can contribute to delay in identification and treatment. The objectives of this report were to identify and describe features that are common in both diagnoses and those that may help distinguish EVALI from MIS-C, and to highlight the diagnostic challenges observed at our tertiary medical center.

Methods: We identified adolescents diagnosed with MIS-C who had respiratory or gastrointestinal symptoms and patients diagnosed with EVALI during the same time period.

A homozygous stop-gain variant in ARHGAP42 is associated with childhood interstitial lung disease, systemic hypertension, and immunological findings.

ARHGAP42 encodes Rho GTPase activating protein 42 that belongs to a member of the GTPase Regulator Associated with Focal Adhesion Kinase (GRAF) family. ARHGAP42 is involved in blood pressure control by regulating vascular tone. Despite these findings, disorders of human variants in the coding part of ARHGAP42 have not been reported.