Developing Assessments for Key Stakeholders in Pediatric Congenital Heart Disease: Qualitative Pilot Study to Inform Designing of a Medical Education Toy.

Background: Congenital heart disease (CHD) is a birth defect of the heart that requires long-term care and often leads to additional health complications. Effective educational strategies are essential for improving health literacy and care outcomes. Despite affecting around 40,000 children annually in the United States, there is a gap in understanding children's health literacy, parental educational burdens, and the efficiency of health care providers in delivering education.

Exploring Health Educational Interventions for Children With Congenital Heart Disease: Scoping Review.

Background: Congenital heart disease (CHD) is the most common birth defect, affecting 40,000 births annually in the United States. Despite advances in medical care, CHD is often a chronic condition requiring continuous management and education. Effective care management depends on children's understanding of their condition.

Disease Burden and Spectrum of Symptoms that Impact Quality of Life in Pediatric Patients with Eosinophilic Esophagitis.

Eosinophilic esophagitis (EoE) is a progressive type 2 inflammatory disease characterized by symptoms related to esophageal dysfunction and significant esophageal eosinophilic infiltration. It can affect patients from infancy through adulthood. Pediatric EoE has a multidimensional impact on the quality of life of both patients and their families.

Evaluation of an engineered vascular graft exhibiting somatic growth in lambs to model repair of absent pulmonary artery branch.

Background: Growth is the holy grail of tissue implants in pediatrics. No vascular graft currently in use for surgical repairs of congenital heart defects has somatic growth capacity.

Methods: Biologically-engineered grafts (6 mm) grown from donor ovine fibroblasts in a sacrificial fibrin gel were implanted into the left pulmonary branch of 3-month old lambs for 3, 6, and 18 months.

Percutaneous Endovascular Stenting of an Acute Complicated Iatrogenic Type B Aortic Dissection in an Infant.

Thoracic endovascular aortic repair has emerged as a viable alternative for managing Sanford type B aortic dissection in adults. We report the first case of managing an acute and evolving communicating type B aortic dissection in an infant with endovascular aortic stenting.

Comparative transcatheter occlusion of patent ductus arteriosus: multicenter collaborative study across pediatric and veterinary cardiology centers.

Introduction/objective: Transcatheter therapeutics have revolutionized treatment of patent ductus arteriosus (PDA). Greater understanding of corrective interventions across species can advance best practices, protocols, and outcomes while minimizing adverse events. The objective of this study was to describe characteristics and outcomes in children and dogs undergoing transcatheter PDA occlusion.

Rationale and design of CHD PULSE: Congenital Heart Disease Project to Understand Lifelong Survivor Experience.

Background: With improved survival of adults with congenital heart disease (CHD) comes a need to understand the lifelong outcomes of this population. The aim of this paper is to describe the rationale and design of Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), a study to determine long-term medical, neurocognitive, and psychosocial outcomes among adults with a history of intervention for CHD and to identify factors associated with those outcomes.

Methods: CHD PULSE is a cross-sectional survey conducted from September 2021 to April 2023 among adults aged 18 and older with a history of at least 1 intervention for CHD at 1 of 11 participating U.

Complex Pulmonary Artery Rehabilitation in Children with Alagille Syndrome: An Early Single-Center Experience of a Successful Collaborative Work.

In this paper, we share our single-center experience of successful multidisciplinary management of patients with Alagille syndrome. In addition, we aim to highlight the need for an Alagille program for effectively managing these patients, in general, and particularly peripheral pulmonary artery stenosis associated with this syndrome. This is a retrospective review of six children with Alagille syndrome and advanced liver involvement who underwent pulmonary artery reconstruction between 2021 and 2022.

Comparative evaluation of bioactivity of MTA plus and MTA plus chitosan conjugate in phosphate buffer saline an invitro study.

Background And Objective: Various materials like MTA, Biodentine etc have been used for the regeneration of lost dental tissues. Still, the quest for newer materials to enhance the bioactivity of the existing materials continues. Hence this study aims at the evaluation of bioactivity of MTA Plus when conjugated with Chitosan in phosphate buffer saline.

Children with eosinophilic esophagitis non-responsive to combination therapy have distinct esophageal transcriptomic and microbiome profile.

Background: A combination of proton-pump inhibitors (PPI) and topical steroids (TS) is used to treat children with eosinophilic esophagitis (EoE). However, a subset of children do not respond to this combination therapy. We aimed to identify the esophageal transcriptional, cell composition, and microbial differences between the non-responders (EoE-PPI-TSnr; n = 7) and responders (EoE-PPI-TSr; n = 7) to the combination therapy for EoE and controls (n = 9) using metatranscriptomics.

Nonepithelial Gene Expression Correlates With Symptom Severity in Adults With Eosinophilic Esophagitis.

Background: The mechanistic basis of the variable symptomatology seen in eosinophilic esophagitis (EoE) remains poorly understood.

Objective: We examined the correlation of a validated, patient-reported outcome metric with a broad spectrum of esophageal transcripts to uncover potential symptom pathogenesis.

Methods: We extracted data from 146 adults with EoE through the Consortium of Eosinophilic Gastrointestinal Disease Researchers.

Clinical and molecular correlates of the Index of Severity for Eosinophilic Esophagitis.

Background: The Index of Severity for Eosinophilic Esophagitis (I-SEE) is a new expert-defined clinical tool that classifies disease severity of eosinophilic esophagitis (EoE).

Objective: We aimed to determine whether I-SEE is associated with patient characteristics, molecular features of EoE, or both.

Methods: We analyzed a prospective cohort of patients with EoE from the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR).

Characterization of lamina propria remodeling in pediatric eosinophilic esophagitis using second harmonic generation microscopy.

Eosinophilic esophagitis (EoE) is a chronic inflammatory condition characterized by an intense infiltration of eosinophils into the esophageal epithelium. When not adequately controlled, eosinophilic inflammation can lead to changes in components of the extracellular matrix (ECM) of the lamina propria. Particularly, alterations to the collagen fiber matrix can lead to lamina propria fibrosis (LPF), which plays an important role in the fibrostenotic complications of EoE.

Recognition and Management of Feeding Dysfunction in the Pediatric Patient with Eosinophilic Esophagitis.

Feeding is a complex skill requiring coordination of multiple body systems. Multiple factors are considered in feeding dysfunction in pediatric patients with eosinophilic gastrointestinal disorders, including overall development, nutritional status, mealtime behaviors, and medical comorbidities. Symptoms of feeding dysfunction vary by age, with maladaptive learned feeding behaviors spanning all age ranges.

Proton Pump Inhibitors Modulate Gene Expression Profile in Esophageal Mucosa and Microbiome.

Objective: Proton pump inhibitors (PPIs) are commonly used to manage children with upper gastrointestinal symptoms and without a formal diagnosis. We investigated the effect of PPIs on esophageal mucosal transcriptome and active microbiota in children with normal esophagi. Furthermore, we examined whether the differences in host esophageal mucosal gene expression were driven by an underlying esophageal epithelial cell type composition.

In Vivo Raman Spectroscopy Reveals Biochemical Composition of the Esophageal Tissue in Pediatric Eosinophilic Esophagitis.

Introduction: Biochemical alterations in the esophagus of patients with eosinophilic esophagitis (EoE) are poorly understood. We used Raman spectroscopy through a pediatric endoscope to identify key Raman features reflective of the esophageal biochemical composition to differentiate between children with EoE from non-EoE controls and between children with active (aEoE) and inactive EoE (iEoE).

Methods: Spectral measurements were obtained using a customized pediatric endoscope-compatible fiber-optic Raman probe in real time during an esophagogastroduodenoscopy.

Transcatheter closure of a large post-myocardial infarction ventricular septal defect using the GORE cardioform ASD Occluder.

A 49-year-old man presented late with an anterior wall myocardial infarction (MI) status post-primary coronary intervention of the left anterior descending artery that resulted in no reflow of the vessel. The patient was transferred to our institution in cardiogenic shock.

A synthesis and subgroup analysis of the eosinophilic esophagitis tissue transcriptome.

Background: Eosinophilic esophagitis (EoE) is a chronic immune mediated inflammatory disorder of the esophagus. It is still unknown why children and adults present differently, and there is little evidence about why it is more common in men than women.

Objective: Our aim was to synthesize published and unpublished esophageal bulk RNA-sequencing (RNA-seq) data to gain novel insights into the pathobiology of EoE and examine the differences in EoE transcriptome by sex and age group.