Integrating Formative and Summative Clinical Skills Examinations to Promote Learning for Early Medical Students: A Mixed Methods Study.

Background: Medical educators aim to train students with high-quality clinical skills through the promotion of self-regulated learning and the development of lifelong learning skills. Formative clinical skills examinations (FCSE) allow for real-time learner-centered feedback and coaching which are key in promoting the development of expertise in early learners. This study assessed the impact of the integration of FCSE with learner-centered, real-time feedback and coaching based on an "educational plan-do-study-act" (PDSA) cycle on early medical students' experience and performance.

Approach to the patient with Childhood Interstitial and Diffuse Lung Disease.

Childhood Interstitial and Diffuse Lung Disease (chILD) encompasses a group of rare, chronic lung disorders in infants and children with overlapping clinical features but diverse etiologies. The clinical presentation of chILD is of chronic or recurring respiratory signs and symptoms, often including increased work of breathing and hypoxia, with diffuse radiographic abnormalities on chest imaging. Recognition can be challenging since some clinical features overlap with those of more common pediatric respiratory diseases including asthma and recurrent viral infections, among others.

Estimating the effect of nintedanib on forced vital capacity in children and adolescents with fibrosing interstitial lung disease using a Bayesian dynamic borrowing approach.

Background: The rarity of childhood interstitial lung disease (chILD) makes it challenging to conduct powered trials. In the InPedILD trial, among 39 children and adolescents with fibrosing ILD, there was a numerical benefit of nintedanib versus placebo on change in forced vital capacity (FVC) over 24 weeks (difference in mean change in FVC % predicted of 1.21 [95% confidence interval: -3.

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.

The lung in inborn errors of immunity: From clinical disease patterns to molecular pathogenesis.

In addition to being a vital organ for gas exchange, the lung is a crucial immune organ continuously exposed to the external environment. Genetic defects that impair immune function, called inborn errors of immunity (IEI), often have lung disease as the initial and/or primary manifestation. Common types of lung disease seen in IEI include infectious complications and a diverse group of diffuse interstitial lung diseases.

Using a Modified Delphi Methodology to Identify Essential Telemedicine Skills for Pediatric Residents.

Objective: Telemedicine use in pediatrics increased during the coronavirus disease-2019 (COVID-19) pandemic. Despite rapid uptake by pediatric residency programs, consensus on essential telemedicine skills for pediatric residents is lacking. We used a modified Delphi methodology to identify essential telemedicine skills and behaviors for pediatric residents.

Pulmonary microbiome and gene expression signatures differentiate lung function in pediatric hematopoietic cell transplant candidates.

Impaired baseline lung function is associated with mortality after pediatric allogeneic hematopoietic cell transplantation (HCT), yet limited knowledge of the molecular pathways that characterize pretransplant lung function has hindered the development of lung-targeted interventions. In this study, we quantified the association between bronchoalveolar lavage (BAL) metatranscriptomes and paired pulmonary function tests performed a median of 1 to 2 weeks before allogeneic HCT in 104 children in The Netherlands. Abnormal pulmonary function was recorded in more than half the cohort, consisted most commonly of restriction and impaired diffusion, and was associated with both all-cause and lung injury-related mortality after HCT.

Pulmonary surveillance in pediatric hematopoietic stem cell transplant: A multinational multidisciplinary survey.

Background: Hematopoietic Stem Cell Transplant (HSCT) is an established treatment for malignant and non-malignant conditions and pulmonary disease is a leading cause of late term morbidity and mortality. Accurate and early detection of pulmonary complications is a critical step in improving long term outcomes. Existing guidelines for surveillance of pulmonary complications post-HSCT contain conflicting recommendations.

NT-proBNP levels and cardiopulmonary function in children with sickle cell disease.

Patients with sickle cell disease (SCD) are living longer and subsequently more apt to develop cardiopulmonary dysfunction. N-terminal pro-brain natriuretic peptide (NT-proBNP) levels have been used in adults with SCD to assess for pulmonary hypertension and mortality. While the incidence of PH is low in pediatrics, it is reasonable to presume that NT-proBNP levels can be used to assess risk for the development of cardiopulmonary morbidity.

Overview of the ChILD Research Network: A roadmap for progress and success in defining rare diseases.

Children's interstitial and diffuse lung diseases are a diverse group of rare lung disorders that present in childhood with diffuse pulmonary infiltrates and respiratory signs and symptoms. Children with these disorders face high morbidity and mortality and their families must cope with overwhelming uncertainty. Physicians caring for these patients are challenged by a paucity of directed therapies, or even understanding of natural history.

Highly Sensitive Blocker Displacement Amplification and Droplet Digital PCR Reveal Low-Level Parental FOXF1 Somatic Mosaicism in Families with Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins.

Detection of low-level somatic mosaicism [alternate allele fraction (AAF) ≤ 10%] in parents of affected individuals with the apparent de novo pathogenic variants enables more accurate estimate of recurrence risk. To date, only a few systematic analyses of low-level parental somatic mosaicism have been performed. Herein, highly sensitive blocker displacement amplification, droplet digital PCR, quantitative PCR, long-range PCR, and array comparative genomic hybridization were applied in families with alveolar capillary dysplasia with misalignment of pulmonary veins.

Identifying Clinical and Research Priorities in Sickle Cell Lung Disease. An Official American Thoracic Society Workshop Report.

Pulmonary complications of sickle cell disease (SCD) are diverse and encompass acute and chronic disease. The understanding of the natural history of pulmonary complications of SCD is limited, no specific therapies exist, and these complications are a primary cause of morbidity and mortality. We gathered a multidisciplinary group of pediatric and adult hematologists, pulmonologists, and emergency medicine physicians with expertise in SCD-related lung disease along with an SCD patient advocate for an American Thoracic Society-sponsored workshop to review the literature and identify key unanswered clinical and research questions.

Thoracic growth deficiency in childhood cancer survivors may cause overestimation of lung disease.

Introduction: Survivors of childhood cancers undergo routine pulmonary function testing as they are at an increased lifetime risk for significant lung disease. However, this population also demonstrates growth abnormalities that could influence the interpretation of these tests, as reference equations are based on standing height. We aim to determine the impact of the relative thoracic growth deficiency in childhood cancer survivors on the interpretation of pulmonary function testing.

Pulmonary Alveolar Proteinosis: A Case of Profound Hypoxemia in a Previously Healthy Teenager.

The profoundly hypoxemic child presents an interesting set of diagnostic and management challenges in the pediatric emergency department. While common pathologies including pneumonia, asthma, bronchiolitis, and pneumothoraces are managed using evidence-based algorithms, more enigmatic pathologies may present the treating physician with less diagnostic and therapeutic clarity. We present the case of a profoundly hypoxemic 16-year-old girl who presented in minimal distress, with oxyhemoglobin saturation of 63% on room air.

Pathogenetics of alveolar capillary dysplasia with misalignment of pulmonary veins.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a lethal lung developmental disorder caused by heterozygous point mutations or genomic deletion copy-number variants (CNVs) of FOXF1 or its upstream enhancer involving fetal lung-expressed long noncoding RNA genes LINC01081 and LINC01082. Using custom-designed array comparative genomic hybridization, Sanger sequencing, whole exome sequencing (WES), and bioinformatic analyses, we studied 22 new unrelated families (20 postnatal and two prenatal) with clinically diagnosed ACDMPV. We describe novel deletion CNVs at the FOXF1 locus in 13 unrelated ACDMPV patients.

Bisphenol A exposure is associated with decreased lung function.

Objective: To examine the associations of bisphenol A (BPA) exposure with lung function measures and exhaled nitric oxide (FeNO) in children.

Study Design: We performed a cross-sectional analysis of a subsample of US children age 6-19 years who participated in the 2007-2010 National Health and Nutrition Examination Survey. We assessed univariate and multivariable associations of urinary BPA concentration with the predicted pulmonary function measures for age, sex, race/ethnicity and height (forced expiratory volume in 1 second [FEV1], forced vital capacity [FVC], forced expiratory flow 25%-75%, and FEV1 divided by FVC) and with FeNO.

Associations of World Trade Center exposures with pulmonary and cardiometabolic outcomes among children seeking care for health concerns.

Objective: Prior research on the physical health of children exposed to the World Trade Center (WTC) attacks has largely relied on parental report via questionnaire. We examined the impact of clinically-reported exposures on the physical health of children who lived and/or attended school in downtown Manhattan on September 11, 2001.

Study Design: We performed a cross-sectional study of 148 patients who presented to the WTC Environmental Health Center/Survivors Health Program, and were ≤ 18 years old on September 11, 2001.

Asthma self-management is sub-optimal in urban Hispanic and African American/black early adolescents with uncontrolled persistent asthma.

Introduction: Youth as young as 11 are given responsibility to manage their asthma. Yet, little is known regarding early adolescents' asthma self-management behaviors. This study characterizes urban early adolescents' asthma self-management behaviors and perceived responsibility to manage asthma, exploring demographic differences and examining the relationship between asthma responsibility and disease management.

Obesity and respiratory diseases in childhood.

The prevalence of childhood obesity has more than tripled over the past five decades. Obesity results in low lung volumes, likely through increased loading of the chest wall and abdomen. The prevalence of asthma in children has paralleled the rise in obesity; obesity may increase the severity of asthma, but a direct link has been difficult to establish.

Cardiopulmonary exercise testing in children and adolescents with asthma who report symptoms of exercise-induced bronchoconstriction.

Patients with asthma often report symptoms of exercise-induced bronchoconstriction. We performed cardiopulmonary exercise testing to establish the cause of exercise limitation in patients with asthma, under treatment, who reported symptoms of exercise-induced bronchoconstriction. Ten of the 42 patients meeting criteria for inclusion in our study (24%) developed exercise-induced bronchoconstriction.