Guidance for stem cell therapy for juvenile systemic sclerosis patients.

Introduction: Autologous stem cell transplantation (ASCT) and cellular therapies (CTs) are emerging therapeutic options for both adult and juvenile-onset systemic sclerosis (jSSc) patients. However, most efficacy data are derived from adult studies, and it remains unclear whether adult stem cell transplant criteria are fully applicable to pediatric patients with jSSc. Given pediatric patients' unique potential for recovery and tissue remodeling, the stringent criteria used in adults need adaptation for children.

Transcriptomic Profiles in Nasal Epithelium and Asthma Endotypes in Youth.

Importance: T helper 2 (T2) cells and T helper 17 (T17) cells are CD4+ T cell subtypes involved in asthma. Characterizing asthma endotypes based on these cell types in diverse groups is important for developing effective therapies for youths with asthma.

Objective: To identify asthma endotypes in school-aged youths aged 6 to 20 years by examining the distribution and characteristics of transcriptomic profiles in nasal epithelium.

Outdoor Air Pollution and Pediatric Respiratory Disease.

Outdoor air pollution is ubiquitous, and no safe level of exposure has been identified for the most common air pollutants such as ozone and particle pollution. Children are uniquely more susceptible to the harms of outdoor air pollution, which can cause and exacerbate respiratory disease. Although challenging to identify the effects of outdoor air pollution on individual patients, understanding the basics of outdoor air pollution is essential for pediatric respiratory health care providers.

Nasal Epithelium Transcriptomics Predict Clinical Response to Elexacaftor/Tezacaftor/Ivacaftor.

Elexacaftor/tezacaftor/ivacaftor (ETI) has had a substantial positive impact for people living with cystic fibrosis (pwCF). However, there can be substantial variability in efficacy, and we lack adequate biomarkers to predict individual response. We thus aimed to identify transcriptomic profiles in nasal respiratory epithelium that predict clinical response to ETI treatment.

Long-Term PM Exposure and Upregulation of Expression in Nasal Epithelium from Youth with Asthma.

Background: Little is known about long-term PM exposure and airway epithelial gene expression.

Objective: To test for association between long-term PM exposure and nasal epithelial gene expression in youth with asthma.

Methods: Transcriptome-wide association study (TWAS) of long-term PM in nasal epithelium from youth aged 6-20 years in the: 1) Epigenetic Variation and Childhood Asthma in Puerto Ricans study (EVA-PR, n=182); 2) Vitamin D Kids Asthma Study (VDKA, n=58); and 3) Stress and Treatment Response in Puerto Rican and African American Children with Asthma study (STAR, n=81).

Persistent cat ownership and asthma in a longitudinal study of Puerto Rican youth.

Background: Epidemiologic studies have reported conflicting findings for cat or dog exposure and childhood asthma. No study has evaluated whether persistent pet exposure from early life to school age is associated with asthma or allergic sensitization in youth.

Objective: To evaluate whether persistent ownership of a cat or a dog throughout childhood is associated with asthma in Puerto Rican youth, a group disproportionately affected with asthma.

Socioeconomic status, diet, and recurrent severe asthma exacerbations in Puerto Rican youth.

Background: Why Puerto Rican youths have higher rates of severe asthma exacerbations (SAEs) than their non-Hispanic White peers is unclear.

Objective: We aimed to identify risk factors associated with recurrent SAEs in Puerto Rican youths with asthma.

Methods: We performed cross-sectional and longitudinal analyses of recurrent SAEs in 209 Puerto Rican youths with asthma who participated in 2 cross-sectional studies approximately 5.

Best clinical practice in the treatment of juvenile systemic sclerosis: expert panel guidance - the result of the International Hamburg Consensus Meeting December 2022.

Introduction: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently there is only one consensus treatment guideline concerning skin, pulmonary and vascular involvement for jSSc, the jSSc SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) initiative, which was based on data procured up to 2014. Therefore, an update of these guidelines, with a more recent literature and expert experience, and extension of the guidance to more aspects of the disease is needed.

Odds of COVID-19-associated asthma exacerbations in children higher during Omicron wave.

Background: We aimed to determine the association of COVID-19 variant wave with asthma exacerbations in children with asthma.

Methods: We conducted a retrospective cross-sectional study of children in the Western Pennsylvania COVID-19 Registry (WPACR). We extracted data for all children in the WPACR with asthma and compared their acute clinical presentation and outcomes during the Pre-Delta (7/1/20-6/30/21), Delta (8/1/21-12/14/21), and Omicron (12/15/21-8/30/22) waves.

Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS).

Background: Juvenile systemic sclerosis (jSSc) is a systemic inflammatory and fibrotic autoimmune disease. Adult guidelines recommend obtaining a screening high-resolution computed tomography scan (CT) at diagnosis. As these recommendations are adopted as standard of care for jSSc, increased screening with CT may lead to increased detection of nodules.

Exposure to violence and asthma in Puerto Rican youth with high Th2 immunity.

Background: Little is known about the determinants of asthma among youth with high T helper 2 (Th2) immunity. We hypothesized that exposure to violence (ETV) and violence-related distress are associated with asthma in children and adolescents with high Th2 immunity.

Methods: We analyzed data from Puerto Ricans with high Th2 immunity aged 9-20 years in the Puerto Rico Genetics of Asthma and Lifestyle (PR-GOAL) and the Epigenetic Variation of Childhood Asthma in Puerto Ricans (EVA-PR) studies, and in a prospective study (PROPRA).

Ozone and childhood respiratory health: A primer for US pediatric providers and a call for a more protective standard.

Ground level ozone is a potent respiratory toxicant with decades of accumulated data demonstrating respiratory harms to children. Despite the ubiquity of ozone in the United States, impacting both urban and rural communities, the associated harms of exposure to this important air pollutant are often infrequently or inadequately covered during medical training including pulmonary specialization. Thus, many providers caring for children's respiratory health may have limited knowledge of the harms which may result in reduced discussion of ozone pollution during clinical encounters.

Air Quality Index and Childhood Asthma: A Pilot Randomized Clinical Trial Intervention.

Introduction: To reduce air pollution exposure, the U.S. asthma guidelines recommend that children check the Air Quality Index before outdoor activity.

Parental knowledge and usage of air quality in childhood asthma management.

Background: The current United States asthma management guidelines recommend usage of the Air Quality Index (AQI) for outdoor activity modification when air pollution is high. Little is known about parental knowledge and usage of air quality including the AQI in managing childhood asthma.

Methods: Forty parents (or legal guardians) of children with persistent asthma completed a questionnaire designed to assess 4 areas related to outdoor air pollution: awareness, perception, behavioral modification, and prior healthcare provider discussion.