Neuroanatomical alterations in young boys and adolescents with Klinefelter syndrome.

Klinefelter syndrome (KS, 47,XXY) is a common sex chromosome aneuploidy in males that is characterized by pubertal developmental delays and a wide range of alterations in cognitive, social and emotional functioning. The neural bases of these behavioral symptoms, however, are unclear. A total of 130 boys and adolescents, including 67 males with KS (11.

Retraction notice to "Neointimal hyperplasia after carotid transection and anastomosis surgery is associated with degradation of decorin and platelet-derived growth factor signaling" [JVS-Vascular Science 2 (2021) 2 - 12].

[This retracts the article DOI: 10.1016/j.jvssci.

Resuscitation arterial waveform quantification and outcomes in pediatric bidirectional Glenn and Fontan patients.

Background: Resuscitation with chest compressions and positive pressure ventilation in Bidirectional Glenn (BDG) or Fontan physiology may compromise passive venous return and accentuate neurologic injury. We hypothesized that arterial pressure and survival would be better in BDG than Fontan patients.

Methods: Secondary analyses of the Pediatric Intensive Care Quality of CPR and Improving Outcomes from Pediatric Cardiac Arrest databases.

Brief report: incidence and outcomes of pediatric tracheal intubation-associated cardiac arrests in the ICU-RESUS clinical trial.

Background: Tracheal intubation (TI)-associated cardiac arrest (TI-CA) occurs in 1.7% of pediatric ICU TIs. Our objective was to evaluate resuscitation characteristics and outcomes between cardiac arrest patients with and without TI-CA.

Impact of Spinal Deformity and Surgery on Health-Related Quality of Life in Cerebral Palsy: A Multicenter Prospective Controlled Trial.

Background: Spinal fusion for scoliosis associated with cerebral palsy (CP) is challenging to study because specialized outcome measures are needed. Therefore, evidence in favor of the benefits of surgery has not been firmly established. This study aimed to determine if corrective spinal fusion improves health-related quality of life (HRQoL) in children with CP scoliosis at 2 years.

Heterologous HSPC Transplantation Rescues Neuroinflammation and Ameliorates Peripheral Manifestations in the Mouse Model of Lysosomal Transmembrane Enzyme Deficiency, MPS IIIC.

Mucopolysaccharidosis III type C (MPS IIIC) is an untreatable neuropathic lysosomal storage disease caused by a genetic deficiency of the lysosomal N-acetyltransferase, HGSNAT, catalyzing a transmembrane acetylation of heparan sulfate. HGSNAT is a transmembrane enzyme incapable of free diffusion between the cells or their cross-correction, which limits development of therapies based on enzyme replacement and gene correction. Since our previous work identified neuroinflammation as a hallmark of the CNS pathology in MPS IIIC, we tested whether it can be corrected by replacement of activated brain microglia with neuroprotective macrophages/microglia derived from a heterologous HSPC transplant.

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.

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.

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.