Low frequency power in cerebral blood flow is a biomarker of neurologic injury in the acute period after cardiac arrest.

Aim: Cardiac arrest often results in severe neurologic injury. Improving care for these patients is difficult as few noninvasive biomarkers exist that allow physicians to monitor neurologic health. The amount of low-frequency power (LFP, 0.

Who Trains the Trainers?: Development of a Faculty Bootcamp for Pediatric Emergency Medicine Resuscitation Procedures.

Objectives: Attending physicians in pediatric emergency medicine (PEM) must be able to perform lifesaving procedures, yet guidelines for maintaining procedural competency do not exist. We implemented a biannual 2-hour "bootcamp" designed to help PEM faculty maintain procedural competency.

Methods: A survey-based needs assessment was used to create a set of goals and objectives for the session and determine which procedural skills to include.

Localized islet nuclear enlargement hyperinsulinism (LINE-HI) due to ABCC8 and GCK mosaic mutations.

Objective: Congenital hyperinsulinism (HI) is the most common cause of persistent hypoglycemia in children. In addition to typical focal or diffuse HI, some cases with diazoxide-unresponsive congenital HI have atypical pancreatic histology termed Localized Islet Nuclear Enlargement (LINE) or mosaic HI, characterized by histologic features similar to diffuse HI, but confined to only a region of pancreas. Our objective was to characterize the phenotype and genotype of children with LINE-HI.

Differentiating Bell's Palsy From Lyme-Related Facial Palsy.

Background And Objectives: To describe the etiology and clinical course of pediatric acute-onset unilateral peripheral facial palsy (FP), to define factors that distinguish Bell's palsy from Lyme-related FP (LRFP), and to determine if early corticosteroid use impacts facial strength recovery in Bell's palsy or LRFP.

Methods: Retrospective cohort study of children 1 to 18 years old who received clinical care within our pediatric clinical care network (Lyme-endemic region) between 2013 and 2018 for acute-onset unilateral peripheral FP.

Results: The study included 306 children; 82 (27%) had LRFP, 209 (68%) had Bell's palsy, and 15 (5%) had FP of different etiology.

Molecular Mechanisms of ARID5B-Mediated Genetic Susceptibility to Acute Lymphoblastic Leukemia.

Background: There is growing evidence for the inherited basis of susceptibility to childhood acute lymphoblastic leukemia (ALL). Genome-wide association studies have identified non-coding ALL risk variants at the ARID5B gene locus, but their exact functional effects and the molecular mechanism linking ARID5B to B-cell ALL leukemogenesis remain largely unknown.

Methods: We performed targeted sequencing of ARID5B in germline DNA of 5008 children with ALL.

Persistence of Chemotherapy-Induced Peripheral Neuropathy Despite Vincristine Reduction in Childhood B-Acute Lymphoblastic Leukemia.

Background: Children with B-acute lymphoblastic leukemia (B-ALL) are at risk for chemotherapy-induced peripheral neuropathy (CIPN). Children's Oncology Group AALL0932 randomized reduction in vincristine and dexamethasone (every 4 weeks vs 12 weeks during maintenance in the average-risk subset of National Cancer Institute standard-B-ALL (SR AR B-ALL). We longitudinally measured CIPN, overall and by treatment group.

Cardiopulmonary Exercise Performance in the Pediatric and Young Adult Population Before and During the COVID-19 Pandemic.

Physical activity (PA) decreased and sedentary behavior (SB) increased in the pediatric population during the Coronavirus Disease 2019 (COVID-19) pandemic. We examined the effects of PA and SB on cardiopulmonary exercise performance in children, adolescents and young adults both with and without underling cardiac disease, and hypothesized that there will be a change in aerobic and physical working capacity during the pandemic. This was a single-center retrospective longitudinal cohort study in patients age 6-22 years who underwent serial maximal cardiopulmonary exercise stress testing before and during the COVID-19 pandemic.

Gene Therapy for Fibrodysplasia Ossificans Progressiva: Feasibility and Obstacles.

Fibrodysplasia ossificans progressiva (FOP) is a rare and devastating genetic disease, in which soft connective tissue is converted into heterotopic bone through an endochondral ossification process. Patients succumb early as they gradually become trapped in a second skeleton of heterotopic bone. Although the underlying genetic defect is long known, the inherent complexity of the disease has hindered the discovery of effective preventions and treatments.

Reduced survival in patients requiring chest tubes with COVID-19 acute respiratory distress syndrome.

Background: Numerous complications requiring tube thoracostomy have been reported among critically ill patients with COVID-19; however, there has been a lack of evidence regarding outcomes following chest tube placement.

Methods: We developed a retrospective observational cohort of all patients admitted to an intensive care unit (ICU) with confirmed COVID-19 to describe the incidence of tube thoracostomy and factors associated with mortality following chest tube placement.

Results: In total, 1705 patients with laboratory confirmed COVID-19 patients were admitted to our ICUs from March 7, 2020, to March 1, 2021, with 69 out of 1705 patients (4.

Ultrasound-Estimated Bladder Weight Correlates With Videourodynamic Studies in Neurogenic Bladder Dysfunction.

Purpose: This retrospective study was designed to evaluate which lower urinary tract ultrasound parameter(s) could predict the results of invasive urodynamic testing which are the current reference standard in the evaluation of bladder dysfunction in children with spina bifida.

Materials And Methods: Fifty eight children with spina bifida undergoing video urodynamic evaluation and a renal bladder ultrasound as their standard of care were evaluated. Quantitative and qualitative ultrasound parameters were then correlated with the videourodynamic study results which served as the reference standard.

The diagnosis and management of Gaucher disease in pediatric patients: Where do we go from here?

Gaucher disease (GD) is an autosomal recessive inherited lysosomal storage disease that often presents in early childhood and is associated with damage to multiple organ systems. Many challenges associated with GD diagnosis and management arise from the considerable heterogeneity of disease presentations and natural history. Phenotypic classification has traditionally been based on the absence (in type 1 GD) or presence (in types 2 and 3 GD) of neurological involvement of varying severity.

Preliminary assessment of paediatric electrophysiology cardiac implantable electronic device resources around the world.

Background: The Pediatric and Congenital Electrophysiology Society (PACES) is a global organisation committed to the care of children and adults with CHD and arrhythmias.

Objective: To evaluate the global needs and potential inequities as it relates to cardiac implantable electronic devices.

Methods: ARROW (Assessment of Rhythm Resources arOund the World) is an online survey about cardiac implantable electronic devices, sent electronically to physicians within the field of Cardiology, Pediatric Cardiology, Electrophysiology and Pediatric Electrophysiology.

Comparison of Visual Acuity Results Between ATS-HOTV and E-ETDRS Testing Methods in Children With Optic Pathway Gliomas.

Purpose: To determine if visual acuity (VA) outcomes are comparable using the amblyopia treatment study HOTV protocol (ATS-HOTV) and electronic Early Treatment of Diabetic Retinopathy Study (E-ETDRS) protocol in children with optic pathway gliomas (OPGs).

Methods: Children enrolled in a prospective study of OPGs were eligible if they completed both the ATS-HOTV and E-ETDRS during the same visit. The contribution of age, testing order, having neurofibromatosis type 1, visual field loss, and circumpapillary retinal nerve fiber layer thickness to VA difference were assessed using generalized estimating equations to account for the intereye correlation.

Institutional Review Board Use of Outside Experts: What Do We Know?

Institutional review boards (IRBs) are permitted by regulation to seek assistance from outside experts when reviewing research applications that are beyond the scope of expertise represented in their membership. There is insufficient understanding, however, of when, why, and how IRBs consult with outside experts, as this practice has not been the primary focus of any published literature or empirical study to date. These issues have important implications for IRB quality.

Corticohippocampal circuit dysfunction in a mouse model of Dravet syndrome.

Dravet syndrome (DS) is a neurodevelopmental disorder due to pathogenic variants in encoding the Nav1.1 sodium channel subunit, characterized by treatment-resistant epilepsy, temperature-sensitive seizures, developmental delay/intellectual disability with features of autism spectrum disorder, and increased risk of sudden death. Convergent data suggest hippocampal dentate gyrus (DG) pathology in DS () mice.

Treatment of Posthemorrhagic Hydrocephalus.

The incidence of intraventricular hemorrhage (IVH) has overall declined to 15% to 20% of preterm infants with birth weight less than 1500 g. One of the major complications of severe IVH is posthemorrhagic ventricular dilation (PHVD). Nearly 10% of all infants with IVH and 20% of infants with severe IVH will develop progressive PHVD requiring surgical intervention to prevent parenchymal damage in the developing brain.

Enhancer retargeting of CDX2 and UBTF::ATXN7L3 define a subtype of high-risk B-progenitor acute lymphoblastic leukemia.

Transcriptome sequencing has identified multiple subtypes of B-progenitor acute lymphoblastic leukemia (B-ALL) of prognostic significance, but a minority of cases lack a known genetic driver. Here, we used integrated whole-genome (WGS) and -transcriptome sequencing (RNA-seq), enhancer mapping, and chromatin topology analysis to identify previously unrecognized genomic drivers in B-ALL. Newly diagnosed (n = 3221) and relapsed (n = 177) B-ALL cases with tumor RNA-seq were studied.

US local public health department spending between 2008 and 2016 did not increase for communities in need.

Background: Greater US local public health department (LPHD) spending has been associated with decreases in population-wide mortality. We examined the association between changes in LPHD spending between 2008 and 2016 and county-level sociodemographic indicators of public health need.

Methods: Multivariable linear regression was used to estimate the association between changes in county-level per-capita LPHD spending and 2008 sociodemographic indicators of interest: percent of population that was over 65 years old, Black, Hispanic, in poverty, unemployed, and uninsured.

Congenital hyperinsulinism in infancy and childhood: challenges, unmet needs and the perspective of patients and families.

Background: Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infants and children, and carries a considerable risk of neurological damage and developmental delays if diagnosis and treatment are delayed. Despite rapid advances in diagnosis and management, long-term developmental outcomes have not significantly improved in the past years. CHI remains a disease that is associated with significant morbidity, and psychosocial and financial burden for affected families, especially concerning the need for constant blood glucose monitoring throughout patients' lives.