Chest compressions for pediatric organized rhythms: A hemodynamic and outcomes analysis.

Aim: Pediatric cardiopulmonary resuscitation (CPR) guidelines recommend starting CPR for heart rates (HRs) less than 60 beats per minute (bpm) with poor perfusion. Objectives were to (1) compare HRs and arterial blood pressures (BPs) prior to CPR among patients with clinician-reported bradycardia with poor perfusion ("BRADY") vs. pulseless electrical activity (PEA); and (2) determine if hemodynamics prior to CPR are associated with outcomes.

Fifteen Years of Spinal Fusion Outcomes in Children With Cerebral Palsy: Are We Getting Better?

Study Design: Retrospective multicenter study.

Objective: We reviewed 15-year trends in operative factors, radiographic and quality of life outcomes, and complication rates in children with cerebral palsy (CP)-related scoliosis who underwent spinal fusion.

Summary Of Background Data: Over the past two decades, significant efforts have been made to decrease complications and improve outcomes of this population.

Derivation of paediatric blood pressure percentiles from electronic health records.

Background: Identification of abnormal blood pressure (BP) in children requires normative data. We sought to examine the feasibility of using "real-world" office BP data obtained from electronic health records (EHR) to generate age-, sex- and height-specific BP percentiles for children.

Methods: Using data collected 01/01/2009-8/31/2021 from eight large children's healthcare organisations in PEDSnet, we applied a mixed-effects polynomial regression model with random slopes to generate Z-scores and BP percentiles and compared them with currently used normative BP distributions published in the 2017 American Academy of Paediatrics (AAP) Clinical Practise Guidelines (CPG).

The Effect of Udenafil on Heart Rate and Blood Pressure in Adolescents With the Fontan Circulation.

The Fontan Udenafil Exercise Longitudinal (FUEL) trial showed that treatment with udenafil was associated with improved exercise performance at the ventilatory anaerobic threshold in children with Fontan physiology. However, it is not known how the initiation of phosphodiesterase 5 inhibitor therapy affects heart rate and blood pressure in this population. These data may help inform patient selection and monitoring after the initiation of udenafil therapy.

School age and adolescent heart failure following the Norwood procedure.

Background: Heart failure results in significant morbidity and mortality for young children with hypoplastic left heart syndrome (HLHS) following the Norwood procedure. The trajectory in later childhood is not well described.

Methods: We studied the outcome into adolescence of participants enrolled in the Single Ventricle Reconstruction trial who underwent the Fontan procedure or survived to 6 years without having undergone Fontan procedure.

Improving the Procedure of Delivering Serious News: Impact of a Six-Month Curriculum for Second Year Pediatric Residents.

We implemented and studied a novel curriculum that combined role play, didactic education, and the use of a procedure card for asynchronous learning to improve second-year pediatric residents' skills in delivering serious news. Phase 1 established baseline performance with a self-efficacy survey and observed simulation delivering serious news. Phase 2 included directed education of participants with a validated communication skills training framework.

A Telemedicine Solution to Minimize Unnecessary Emergency Department Transfers for Low-acuity Pediatric Orthopaedic Patients: A Model for Cost Minimization.

Introduction: Unnecessary emergency department (ED) transfers represent a notable source of excess costs and misutilization of healthcare resources, particularly with management of acute pediatric musculoskeletal injuries. This study used institutional data to create a model investigating the expected costs of a formal peer-to-peer telemedicine intervention designed to triage pediatric orthopaedic transfers, which we hypothesized would decrease healthcare costs by minimizing unwarranted ED-to-ED transfers.

Methods: In this retrospective modeling analysis, 350 pediatric orthopaedic trauma patients transferred to two in-network referral hospitals from outside facilities were identified and stratified into three groups representing how patients theoretically optimally could have been treated.

Cognition, Academic Achievement, Adaptive Behavior, and Quality of Life in Child and Adolescent Boys with Klinefelter Syndrome.

Objective: Klinefelter syndrome (KS; 47, XXY), the most common sex chromosome aneuploidy in males, is characterized by testicular failure and testosterone deficiency as well as a variety of cognitive, social, and emotional challenges. In the current study, we aimed to clarify the cognitive-behavioral profile of peripubertal boys with KS using measures of cognition, academic achievement, adaptive behavior, and quality of life.

Method: We compared 47 boys with KS (7-16 years of age) with 55 performance IQ-matched boys without KS on measures of cognition (WISC-V), executive function (BRIEF-2), academic achievement (KTEA-3), adaptive behavior (Vineland-3), and quality of life (PROMIS).

Association of CPR simulation program characteristics with simulated and actual performance during paediatric in-hospital cardiac arrest.

Aim: To evaluate associations between characteristics of simulated point-of-care cardiopulmonary resuscitation (CPR) training with simulated and actual intensive care unit (ICU) CPR performance, and with outcomes of children after in-hospital cardiac arrest.

Methods: This is a pre-specified secondary analysis of the ICU-RESUScitation Project; a prospective, multicentre cluster randomized interventional trial conducted in 18 ICUs from October 2016-March 2021. Point-of-care bedside simulations with real-time feedback to allow multidisciplinary ICU staff to practice CPR on a portable manikin were performed and quality metrics (rate, depth, release velocity, chest compression fraction) were recorded.

Echocardiographic image collection and evaluation in infants with CHD: lessons learned from the imaging core lab for the Residual Lesion Score study.

Many factors affect patient outcome after congenital heart surgery, including the complexity of the heart disease, pre-operative status, patient specific factors (prematurity, nutritional status and/or presence of comorbid conditions or genetic syndromes), and post-operative residual lesions. The esidual esion core is a novel tool for assessing whether specific residual cardiac lesions after surgery have a measurable impact on outcome. The goal is to understand which residual lesions can be tolerated and which should be addressed prior to leaving the operating room.

Molecular Trojan Horses for treating lysosomal storage diseases.

Lysosomal storage diseases (LSDs) are caused by monogenic mutations in genes encoding for proteins related to the lysosomal function. Lysosome plays critical roles in molecule degradation and cell signaling through interplay with many other cell organelles, such as mitochondria, endoplasmic reticulum, and peroxisomes. Even though several strategies (i.

Natural History of Pediatric Hand and Wrist Ganglion Cysts: Longitudinal Follow-Up of a Prospective, Dual-Center Cohort.

Purpose: This investigation describes the outcomes of pediatric ganglion cysts in a prospective cohort that elected not to undergo cyst aspiration or surgical treatment. Our primary aim was to investigate the rate of spontaneous resolution over time among the subset of patients who did not undergo specific treatments.

Methods: Children (aged ≤18 years) who presented to the clinic with ganglion cysts of the hand or wrist were enrolled in a prospective two-center registry between 2017 and 2021.

Intraoperative neuromonitoring predicts postoperative deficits in severe pediatric spinal deformity patients.

Purpose: To evaluate intraoperative monitoring (IOM) alerts and neurologic deficits during severe pediatric spinal deformity surgery.

Methods: Patients with a minimum Cobb angle of 100° in any plane or a scheduled vertebral column resection (VCR) with minimum 2-year follow-up were prospectively evaluated (n = 243). Preoperative, immediate postoperative, and 2-year postoperative neurologic status were reported.

More Than a Headache: Lived Experience of Migraine in Youth.

Background: Migraine is the leading cause of disability among adolescents and young adults. We aimed to characterize the impact of migraine on the experience of children, adolescents, and caregivers.

Methods: This descriptive qualitative study recruited youth aged four to 18 years with migraine and their caregivers from the multicenter, prospective Pediatric Migraine Registry between 2020 and 2021.

The Fontan Udenafil Exercise Longitudinal Trial: Subgroup Analysis.

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.

Clinical course and management of children with IgA vasculitis with nephritis.

Background: IgA vasculitis is the most common vasculitis in children and is often complicated by acute nephritis (IgAVN). Risk of chronic kidney disease (CKD) among children with IgAVN remains unknown. This study aimed to describe the clinical management and kidney outcomes in a large cohort of children with IgAVN.

Valosin-containing protein (VCP/p97) is prognostically unfavorable in pediatric AML, and negatively correlates with unfolded protein response proteins IRE1 and GRP78: A report from the Children's Oncology Group.

Purpose: The endoplasmic reticulum (ER) is the major site of protein synthesis and folding in the cell. ER-associated degradation (ERAD) and unfolded protein response (UPR) are the main mechanisms of ER-mediated cell stress adaptation. Targeting the cell stress response is a promising therapeutic approach in acute myeloid leukemia (AML).

Sedation and Analgesia for Reduction of Pediatric Ileocolic Intussusception.

Importance: Ileocolic intussusception is an important cause of intestinal obstruction in children. Reduction of ileocolic intussusception using air or fluid enema is the standard of care. This likely distressing procedure is usually performed without sedation or analgesia, but practice variation exists.

Characteristics and prognostic impact of IDH mutations in AML: a COG, SWOG, and ECOG analysis.

Somatic mutations in isocitrate dehydrogenase (IDH) genes occur frequently in adult acute myeloid leukemia (AML) and less commonly in pediatric AML. The objective of this study was to describe the prevalence, mutational profile, and prognostic significance of IDH mutations in AML across age. Our cohort included 3141 patients aged between <1 month and 88 years treated on Children's Cancer Group/Children's Oncology Group (n = 1872), Southwest Oncology Group (n = 359), Eastern Cooperative Oncology Group (n = 397) trials, and in Beat AML (n = 333) and The Cancer Genome Atlas (n = 180) genomic characterization cohorts.

A Sonography-based Decision Model for Use in Treatment of Developmental Dysplasia of the Hip.

The objective of this study was to delineate a model for management of developmental dysplasia of the hip (DDH) treatment that incorporates hip ultrasound with objective/predicative parameters at key decision-making times. Hip sonograms of 74 infants (59 females, 15 males; 141 hips) with DDH were retrospectively reviewed. Hip sonographic score (HSS; ranges 0-10) was developed to reflect hip status based on sonographic position, stability, and morphology.

The Risk of Malignant Degeneration of Müllerian Derivatives in PMDS: A Review of the Literature.

Persistent Müllerian Duct Syndrome (PMDS) is a rare autosomal recessive disorder of sex development characterized by the presence of fallopian tubes, uterus and upper one-third of the vagina in individuals with XY genotype and normal male phenotype. The main complications of PMDS are infertility and the rare risk of malignant degeneration of both testicular and Müllerian derivatives. We report the case of a 49-year-old man who, during repair of an incisional hernia, was incidentally found to have a uterine-like structure posterior to the bladder.

Perioperative Complications and Health-related Quality of Life Outcomes in Severe Pediatric Spinal Deformity.

Study Design: Prospective multicenter cohort study.

Objective: To evaluate perioperative complications and mid-term outcomes for severe pediatric spinal deformity.

Summary Of Background Data: Few studies have evaluated the impact of complications on health-related quality of life (HRQoL) outcomes in severe pediatric spinal deformity.

LHX2 haploinsufficiency causes a variable neurodevelopmental disorder.

Purpose: LHX2 encodes the LIM homeobox 2 transcription factor (LHX2), which is highly expressed in brain and well conserved across species, but it has not been clearly linked to neurodevelopmental disorders (NDDs) to date.

Methods: Through international collaboration, we identified 19 individuals from 18 families with variable neurodevelopmental phenotypes, carrying a small chromosomal deletion, likely gene-disrupting or missense variants in LHX2. Functional consequences of missense variants were investigated in cellular systems.