The effect of positive end-expiratory pressure on cardiac output and oxygen delivery during cardiopulmonary resuscitation.

Background: Positive end-expiratory pressure (PEEP) is used to optimize oxygenation by preventing alveolar collapse. However, PEEP can potentially decrease cardiac output through cardiopulmonary interactions. The effect of PEEP on cardiac output during cardiopulmonary resuscitation (CPR) is not known.

Sudden cardiac death in children and young adults without structural heart disease: a comprehensive review.

Sudden cardiac death (SCD) is a rare clinical encounter in pediatrics, but its social impact is immense because of its unpredicted and catastrophic nature in previously healthy individuals. Unlike in adults where the primary cause of SCD is related to ischemic heart disease, the etiology is diverse in young SCD victims. Although certain structural heart diseases may be identified during autopsy in some SCD victims, autopsy-negative SCD is more common in pediatrics, which warrants the diagnosis of sudden arrhythmic death syndrome (SADS) based upon the assumption that the usual heart rhythm is abruptly replaced by lethal ventricular arrhythmia.

Use of pulse contour technology for continuous blood pressure monitoring in pediatric patients.

Objectives: This study evaluates the accuracy of continuous blood pressure monitoring using pulse contour technology with the ClearSight monitoring device, a noninvasive alternative to placing an invasive arterial line, in pediatric patients.

Methods: Children younger than 18 years admitted to a pediatric ICU, who required an arterial line, and fit into the ClearSight finger cuff were included. Blood pressure measurement for systolic, diastolic, and mean arterial pressures (MAP) obtained by the ClearSight device were compared with those obtained with the intra-arterial catheter as well as automated cuff measurements using the mixed-effects model.

The Resource Use Inflection Point for Safe NICU Discharge.

Objectives: (1) To identify a resource use inflection point (RU-IP) beyond which patients in the NICU no longer received NICU-level care, (2) to quantify variability between hospitals in patient-days beyond the RU-IP, and (3) to describe risk factors associated with reaching an RU-IP.

Methods: We evaluated infants admitted to any of the 43 NICUs over 6 years. We determined the day that each patient's total daily standardized cost was <10% of the mean first-day NICU room cost and remained within this range through discharge (RU-IP).

Paediatric Strategy Forum for medicinal product development for acute myeloid leukaemia in children and adolescents: ACCELERATE in collaboration with the European Medicines Agency with participation of the Food and Drug Administration.

Purpose: The current standard-of-care for front-line therapy for acute myeloid leukaemia (AML) results in short-term and long-term toxicity, but still approximately 40% of children relapse. Therefore, there is a major need to accelerate the evaluation of innovative medicines, yet drug development continues to be adult-focused. Furthermore, the large number of competing agents in rare patient populations requires coordinated prioritisation, within the global regulatory framework and cooperative group initiatives.

Understanding the Mechanisms by Which Epigenetic Modifiers Avert Therapy Resistance in Cancer.

The development of resistance to anti-cancer therapeutics remains one of the core issues preventing the improvement of survival rates in cancer. Therapy resistance can arise in a multitude of ways, including the accumulation of epigenetic alterations in cancer cells. By remodeling DNA methylation patterns or modifying histone proteins during oncogenesis, cancer cells reorient their epigenomic landscapes in order to aggressively resist anti-cancer therapy.

Molecular characterization of the calcium release channel deficiency syndrome.

We identified a potentially novel homozygous duplication involving the promoter region and exons 1-4 of the gene encoding type 2 cardiac ryanodine receptor (RYR2) that is responsible for highly penetrant, exertion-related sudden deaths/cardiac arrests in the Amish community without an overt phenotype to suggest RYR2-mediated catecholaminergic polymorphic ventricular tachycardia (CPVT). Homozygous RYR2 duplication (RYR2-DUP) induced pluripotent stem cell cardiomyocytes (iPSC-CMs) were generated from 2 unrelated patients. There was no difference in baseline Ca2+ handling measurements between WT-iPSC-CM and RYR2-DUP-iPSC-CM lines.

The Cost and Educational Experience of Treating Supracondylar Humerus Fractures: A Pilot Analysis on Standardizing Surgical Care.

Unlabelled: Closed reduction and percutaneous pinning (CRPP) for supracondylar humeral fractures (SCHF) comprised considerable surgical volume in pediatric orthopaedics. Limited reports are available on how standardization of the surgical care affects the cost and trainee's learning experience.

Methods: Cost analysis was performed by chart review with the billing department in a university teaching hospital.

Fathers of Children With Congenital Heart Disease: Sources of Stress and Opportunities for Intervention.

Objectives: To examine sources of stress for fathers of children with congenital heart disease and opportunities for intervention to prevent or reduce paternal mental health problems.

Design: Qualitative study using online crowdsourcing, an innovative research methodology to create an online community to serve as a research sample.

Setting: Yammer, an online social networking site.

Germline Sequencing Identifies Rare Variants in Finnish Subjects with Familial Germ Cell Tumors.

Purpose: Pediatric germ cell tumors are rare, representing about 3% of childhood malignancies in children less than 15 years of age, presenting in neonates or adolescents with a greater incidence noted in older adolescents. Aberrations in primordial germ cell proliferation/differentiation can lead to a variety of neoplasms, including teratomas, embryonal carcinoma, choriocarcinoma, and yolk sac tumors.

Patients And Methods: Three Finnish families with varying familial germ cell tumors were identified, and whole-genome sequencing was performed using an Illumina sequencing platform.

Machine learning for automatic identification of thoracoabdominal asynchrony in children.

Background: The current methods for assessment of thoracoabdominal asynchrony (TAA) require offline analysis on the part of physicians (respiratory inductance plethysmography (RIP)) or require experts for interpretation of the data (sleep apnea detection).

Methods: To assess synchrony between the thorax and abdomen, the movements of the two compartments during quiet breathing were measured using pneuRIP. Fifty-one recordings were obtained: 20 were used to train a machine-learning (ML) model with elastic-net regularization, and 31 were used to test the model's performance.

Multisystem Inflammatory Syndrome in Children (MIS-C):: An Emerging Immune Mediated Syndrome in Children Associated with COVID-19.

As the world came to terms with the current COVID-19 pandemic, children were initially thought to have milder disease than adults with significantly lower morbidity and mortality. The emergence of a multi-system inflammatory syndrome in children (MIS-C) associated with the SARS-CoV-2 virus was first recognized in Europe, and then across centers in the United States. Early and widespread data sharing among centers across the world was extremely helpful in early identification and treatment of these children, with a good prognosis in a majority of cases.

Perspectives on Molecular Diagnostic Testing for the COVID-19 Pandemic in Delaware.

The United States has quickly transitioned into one of the epicenters for the coronavirus pandemic. Limitations for rapid testing for the virus responsible for the pandemic, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is the single most important barrier for early detection and prevention of future outbreaks. Combining innovative molecular biology techniques, such as clustered regularly interspaced short palindromic repeats (CRISPR)/Cas nuclease systems and next generation sequencing (NGS) may prove to be an effective solution to establish a high-throughput diagnostic and genomic surveillance workflow for COVID-19 in the State of Delaware.

Extracorporeal Life Support Organization Coronavirus Disease 2019 Interim Guidelines: A Consensus Document from an International Group of Interdisciplinary Extracorporeal Membrane Oxygenation Providers.

Disclaimer: The Extracorporeal Life Support Organization (ELSO) Coronavirus Disease 2019 (COVID-19) Guidelines have been developed to assist existing extracorporeal membrane oxygenation (ECMO) centers to prepare and plan provision of ECMO during the ongoing pandemic. The recommendations have been put together by a team of interdisciplinary ECMO providers from around the world. Recommendations are based on available evidence, existing best practice guidelines, ethical principles, and expert opinion.

Successful use of transarterial radioembolization with yttrium-90 (TARE-Y90) in two children with hepatoblastoma.

Primary malignant liver tumors are rare but all require surgical resection as part of therapy with curative intent. A minority of patients have resectable tumors at diagnosis. Chemotherapy has a therapeutic role in hepatoblastoma but only one-third of patients have resectable disease at diagnosis.

Laboratory Testing Methods for Novel Severe Acute Respiratory Syndrome-Coronavirus-2 (SARS-CoV-2).

Following the first reports of coronavirus disease-19 (COVID-19) by China to the World Health Organization (WHO) on 31st December 2019, more than 4,302,774 novel severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) cases have been reported by authorities in 212 countries and territories by 12th May 2020. The outbreak and spread of COVID-19 worldwide, highlights the critical need for developing rapid and accurate diagnostic testing methods for emerging human coronavirus (CoV) infections. Testing is crucial to track the spread of disease during a pandemic, and to swiftly permit public health interventions including isolation, quarantine, and appropriate clinical management of afflicted individuals.

Subcutaneous emphysema following dental care of a patient with ventriculoperitoneal shunt: Case report.

Introduction: Pediatric dental treatment utilizing an air-driven handpiece and triplex syringe in the operating room has been common practice for many decades. Adverse effects with compressed air are rarely reported in the dental community but can cause serious complications for pediatric patients in the unlikely event they occur.

Case Report: We present a case of subcutaneous emphysema of a 4-year-old presenting for dental rehabilitation under general anesthesia, which was further complicated by the presence of a ventriculoperitoneal (VP) shunt and the patient's neuroatypical status.

Outcomes of pregnancy in operative vs. nonoperative adolescent idiopathic scoliosis patients at mean 30-year follow-up.

Study Design: Retrospective cohort study.

Objective: To determine whether patients who underwent surgical treatment of adolescent idiopathic scoliosis (AIS) in childhood would have an increased C-section risk in adulthood. Although the impact of scoliosis on future pregnancy and child delivery is a common question for patients and parents, there is limited data regarding pregnancy outcomes following childhood treatment of AIS.

Liver-Targeted AAV8 Gene Therapy Ameliorates Skeletal and Cardiovascular Pathology in a Mucopolysaccharidosis IVA Murine Model.

Mucopolysaccharidosis type IVA (MPS IVA) is due to the deficiency of GALNS (-acetylgalactosamine 6-sulfate sulfatase) and is characterized by systemic skeletal dysplasia. We have evaluated adeno-associated virus 8 (AAV8) vectors expressing different forms of human GALNS under a liver-specific promoter. The vectors were delivered intravenously into 4-week-old MPS IVA knockout (KO) and immune tolerant (MTOL) mice at a dose of 5 × 10 genome copies (GC)/kg.

Estimating the relative frequency of leukodystrophies and recommendations for carrier screening in the era of next-generation sequencing.

Leukodystrophies are a heterogeneous group of heritable disorders characterized by abnormal brain white matter signal on magnetic resonance imaging (MRI) and primary involvement of the cellular components of myelin. Previous estimates suggest the incidence of leukodystrophies as a whole to be 1 in 7,000 individuals, however the frequency of specific diagnoses relative to others has not been described. Next generation sequencing approaches offer the opportunity to redefine our understanding of the relative frequency of different leukodystrophies.

Lessons Learned: Identifying Items Felt To Be Critical to Leading a Pediatric Palliative Care Program in the Current Era of Program Development.

The experience of starting and growing a pediatric palliative care program (PPCP) has changed over the last 10 years as rapid increases of patient volume have amplified challenges related to staffing, funding, standards of practice, team resilience, moral injury, and burnout. These challenges have stretched new directors' leadership skills, yet, guidance in the literature on identifying and managing these challenges is limited. A convenience sample of 15 PPCP directors who assumed their duties within the last 10 years were first asked the following open-ended question: What do you wish you had known before starting or taking over leadership of a PPCP? Responses were grouped into themes based on similarity of content.

What's New in the Orthopaedic Treatment of Ambulatory Children With Cerebral Palsy Using Gait Analysis.

Background: Limb deformities in ambulatory children with cerebral palsy (CP) are common. The natural history of lower extremity deformities is variable and the impact on gait is managed with many treatment modalities. Effective interventions must consider the underlying pathophysiology, patient-specific goals, and incorporate objective outcome assessment.

Reducing resource utilization for patients with uncomplicated appendicitis through use of same-day discharge and elimination of postoperative antibiotics.

Background: There is controversy over certain aspects of post-appendectomy care for children with uncomplicated appendicitis. Some institutions have embraced the practice of same-day discharge after appendectomy, while others are hesitant due to concerns about increased readmissions or emergency department (ED) visits. Similarly, some surgeons have transitioned to treating gangrenous appendicitis with a single perioperative dose, while others are concerned about increased risk of infection in this population.

Pediatric Heatstroke Fatalities Caused by Being Left in Motor Vehicles.

Objectives: Fatalities due to being left in motor vehicles is an important cause of pediatric mortality. Few studies in the medical literature focus on this topic. This study aims to describe the circumstances surrounding these deaths, to determine their geographic distribution, and to evaluate the legal consequences for those responsible.