Building a dedicated pediatric pulmonary hypertension program: A consensus statement from the pediatric pulmonary hypertension network.

Pediatric pulmonary hypertension (PH) is a severe, life-threatening disease associated with diverse cardiac, pulmonary, and systemic disorders, which generally requires expertise from multiple disciplines for management. Unfortunately, expert centers are limited, often due to inadequate resources or unfamiliarity with needed components for success. The Pediatric Pulmonary Hypertension Network (PPHNet) includes expert centers in North America specifically dedicated to advancing the field of pediatric PH through research and excellent clinical care.

The Liver Biopsy in Neonatal Cholestasis: Just a Cherry on Top?

Content available: Author Audio Recording.

Managing acute pain in children presenting to the emergency department without opioids.

Pediatric pain is challenging to assess and manage. Frequently underestimated in children, untreated pain may have consequences including increased fear, anxiety, and psychological issues. With the current opioid crisis, emergency physicians must be knowledgeable in both pharmacologic and non-pharmacologic approaches to address pain and anxiety in children that lead to enhanced patient cooperation and family satisfaction.

Are personality disorders in bipolar patients more frequent in the US than Europe?

Objective: Bipolar patients in the United States (US) compared to those from the Netherlands and Germany (here abbrev. as "Europe") have more Axis I comorbidities and more poor prognosis factors such as early onset and psychosocial adversity in childhood. We wished to examine whether these differences also extended to Axis II personality disorders (PDs).

Going viral: A scoping review of the current state and impact of online research dissemination in emergency medicine.

Background: The use of free open-access medical education (FOAM) and other online knowledge dissemination methods has increased over the past decade. However, the role and impact of these tools in the knowledge translation continuum are poorly understood, potentially limiting the ability of knowledge generators to fully harness and exploit their potential. Here, we aim to comprehensively map and synthesize the literature describing the use of online tools for the dissemination of emergency medicine research.

Exome sequencing identified a novel HIST1H1E heterozygous protein-truncating variant in a 6-month-old male patient with Rahman syndrome: A case report.

Rahman syndrome is a rare congenital anomaly syndrome recently described, which results from pathogenic variants in the HIST1H1E gene. The condition is characterized by variable somatic overgrowth, macrocephaly, distinctive facial features, intellectual disability, and behavioral problems. This report extends the genotype and clinical phenotype of HIST1H1E-associated Rahman syndrome.

Validity evidence for an instrument for cognitive load for virtual didactic sessions.

Background: COVID necessitated the shift to virtual resident instruction. The challenge of learning via virtual modalities has the potential to increase cognitive load. It is important for educators to reduce cognitive load to optimize learning, yet there are few available tools to measure cognitive load.

Facilitating health promoting ideas and actions: participatory research in an underserved Swedish residential area.

Background: For an intervention to contribute to decreased health gaps, people living in underserved areas must participate in the research-to-action process during the development of the intervention. The purpose of this study was to collaborate with residents living in a Swedish underserved area to generate health and wellness priorities and actions.

Methods: We applied Group Level Assessment (GLA) together with people living in a Swedish neighborhood where obesity, dental caries and other illnesses are prevalent.

Video screen visualization patterns when using a video laryngoscope for tracheal intubation: A systematic review.

Objective: Published studies of video laryngoscopes are often limited by the lack of a clear definition of video laryngoscopy (VL). We performed a systematic review to determine how often published studies of VL report on video screen visualization.

Methods: We searched PubMed, EMBASE and Scopus for interventional and observational studies in which a video laryngoscope equipped with a standard geometry blade was used for tracheal intubation.

Predictors of Glycemic Outcomes at 1 Year Following Pediatric Total Pancreatectomy With Islet Autotransplantation.

Objective: Total pancreatectomy with islet autotransplantation (TPIAT) is indicated to alleviate debilitating pancreas-related pain and mitigate diabetes in patients with acute recurrent and chronic pancreatitis when medical/endoscopic therapies fail. Our aim was to evaluate predictors of insulin requirement at 1 year following TPIAT in a cohort of children.

Research Design And Methods: This was a review of 43 pediatric patients followed after TPIAT for 1 year or longer.

Preterm infant with diprosopus and holoprosencephaly.

Diprosopus is an extremely rare congenital anomaly involving craniofacial duplication. The etiology and pathophysiology remain unknown, and no genetic mutations have been definitively associated with the condition. This case describes an infant born at 27-weeks completed gestation with multiple congenital anomalies including diprosopus and discusses the implications of prenatal diagnosis.

Procedural Risk in Congenital Cardiac Catheterization (PREDICT).

Background Advancements in the field, including novel procedures and multiple interventions, require an updated approach to accurately assess patient risk. This study aims to modernize patient hemodynamic and procedural risk classification through the creation of risk assessment tools to be used in congenital cardiac catheterization. Methods and Results Data were collected for all cases performed at sites participating in the C3PO (Congenital Cardiac Catheterization Project on Outcomes) multicenter registry.

Utilization and effectiveness of the organ procurement and transplantation network "safety-net" policy.

Background: Liver transplant recipients with persistent renal dysfunction may be prioritized on the kidney transplant waitlist based on the Organ Procurement and Transplantation Network "safety-net" policy implemented in 2017. The aim of this study was to evaluate the utilization of kidney transplant and posttransplant outcomes, of liver transplant recipients with persistent renal dysfunction before and after implementation of the Organ Procurement and Transplantation Network kidney safety-net policy and standardization of simultaneous liver-kidney requirements.

Methods: Using the United Network for Organ Sharing database from January 2015 to March 2019, outcomes of liver transplant recipients listed for kidney transplant and the subset who received kidney after liver transplants were compared before and after policy implementation.

Psychotropic Drug Treatment Patterns in Persons with Fragile X Syndrome.

Psychiatric comorbidity is common in fragile X syndrome (FXS) and often addressed through pharmacological management. Here we examine data in the Fragile X Online Registry With Accessible Research Database (FORWARD) to characterize specific symptoms being treated with psychotropic medication, patterns of medication use, as well as the influence of gender, intellectual disability (ID), age, and autism spectrum disorder (ASD) diagnosis. Data were drawn from the 975 participants who have a completed clinician form.

Vascular Smooth Muscle Cells in Aortic Aneurysm: From Genetics to Mechanisms.

Aortic aneurysm, including thoracic aortic aneurysm and abdominal aortic aneurysm, is the second most prevalent aortic disease following atherosclerosis, representing the ninth-leading cause of death globally. Open surgery and endovascular procedures are the major treatments for aortic aneurysm. Typically, thoracic aortic aneurysm has a more robust genetic background than abdominal aortic aneurysm.

Angiostatic Peptide, Endostatin, Predicts Severity in Pediatric Congenital Heart Disease-Associated Pulmonary Hypertension.

Background Endostatin, an angiogenic inhibitor, is associated with worse pulmonary arterial hypertension (PAH) outcomes in adults and poor lung growth in children. This study sought to assess whether endostatin is associated with disease severity and outcomes in pediatric PAH. Methods and Results Serum endostatin was measured in cross-sectional (N=160) and longitudinal cohorts (N=64) of pediatric subjects with PAH, healthy pediatric controls and pediatric controls with congenital heart disease (CHD) (N=54, N=15), and adults with CHD associated PAH (APAH-CHD, N=185).

The Effect of Adiposity on Cardiovascular Function and Myocardial Fibrosis in Patients With Duchenne Muscular Dystrophy.

Background Patients with Duchenne muscular dystrophy (DMD) develop cardiomyopathy because of a dystrophin deficiency causing fibrofatty replacement of the myocardium. Corticosteroid use and mobility limitations place these patients at risk for increased adiposity. We sought to determine the association of adiposity with cardiovascular dysfunction in patients with DMD.

Termination Based on Event Accrual in Per Protocol Versus Intention to Treat in the ROCKET AF Trial.

Background In event-driven clinical trials, study termination is based on accrual of a target number of primary efficacy events. For noninferiority trials in which superiority is conditionally examined, the ideal cohort in which to track event accrual is unclear. We used data from the ROCKET AF (Rivaroxaban Once Daily Oral Direct Factor Xa Inhibition Compared With Vitamin K Antagonism for Prevention of Stroke and Embolism Trial in Atrial Fibrillation) trial to determine the effect of primary efficacy-event tracking in the per-protocol cohort during the on-treatment period versus the intention-to-treat (ITT) cohort during the ITT period.

Pediatric emergency medicine fellowship point-of-care ultrasound training in 2020.

Objectives: The primary objective was to survey pediatric emergency medicine (PEM) leaders and fellows regarding point-of-care ultrasound (POCUS) training in PEM fellowship programs, including teaching methods, training requirements, and applications taught. Secondary objectives were to compare fellows' and program leaders' perceptions of fellow POCUS competency and training barriers.

Methods: This was a cross-sectional survey of U.

Risk Assessment of the Door-In-Door-Out Process at Primary Stroke Centers for Patients With Acute Stroke Requiring Transfer to Comprehensive Stroke Centers.

Background Patients with acute stroke at non- or primary stroke centers (PSCs) are transferred to comprehensive stroke centers for advanced treatments that reduce disability but experience significant delays in treatment and increased adjusted mortality. This study reports the results of a proactive, systematic, risk assessment of the door-in-door-out process and its application to solution design. Methods and Results A learning collaborative (clinicians, patients, and caregivers) at 2 PSCs and 3 comprehensive stroke centers in Chicago, Illinois participated in a failure modes, effects, and criticality analysis to identify steps in the process; failures of each step, underlying causes; and to characterize each failure's frequency, impact, and safeguards using standardized scores to calculate risk priority and criticality numbers for ranking.