Body Composition in Preterm Infants: Current Insights and Emerging Perspectives.

In recent years, significant advancements in respiratory and nutritional care have markedly improved the survival rates of preterm infants and enhanced long-term health outcomes. Despite these improvements, emerging research highlights the lasting impacts of early growth patterns on an individual's health trajectory. Adults born prematurely face a higher incidence of health issues related to their early birth.

High clinical burden of classical homocystinuria in the United States: a retrospective analysis.

Background: Classical homocystinuria (HCU) is a rare genetic metabolic disorder resulting in elevated homocysteine and methionine levels. The clinical characteristics and associated complications of HCU are well documented. However, there is limited published research on the clinical burden of patients with HCU, especially stratified by total homocysteine (tHcy) levels.

Practice patterns for acquiring neuroimaging after pediatric in-hospital cardiac arrest.

Aims: To determine which patient and cardiac arrest factors were associated with obtaining neuroimaging after in-hospital cardiac arrest, and among those patients who had neuroimaging, factors associated with which neuroimaging modality was obtained.

Methods: Retrospective cohort study of patients who survived in-hospital cardiac arrest (IHCA) and were enrolled in the ICU-RESUS trial (NCT02837497).

Results: We tabulated ultrasound (US), CT, and MRI frequency within 7 days following IHCA and identified patient and cardiac arrest factors associated with neuroimaging modalities utilized.

Evolution of sleep disordered breathing in infants with achondroplasia.

Purpose: Children with achondroplasia (ACH) are at risk for sudden death in infancy due to sleep disordered breathing (SDB) and foramen magnum stenosis (FMS). Sleep studies and neuroimaging are performed in infants with ACH, but interpretation of infant studies is challenging. We sought to describe baseline data on polysomnography (PSG) indices in infants with achondroplasia as well as effects of age and surgery on these parameters.

The ACTION VAD Registry: A Collective Five-Year Experience.

Background: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) began in 2018 as a collaborative learning health system committed to improving outcomes in pediatric heart failure, including children and adults with congenital heart disease, supported with ventricular assist devices (VADs). This report describes patient and device characteristics, and outcomes through 1-year post-implant.

Methods: The ACTION VAD registry report was created from data submitted to the ACTION learning network from April 2018-June 2023.

Social, Emotional, and Behavioral Functioning in Adolescents with Klinefelter Syndrome.

Objective: Klinefelter syndrome (KS) is a common genetic condition in males associated with an extra X chromosome (i.e., 47,XXY).

Increased usage of doxycycline for young children with Lyme disease.

Background: The 2018 Infectious Disease Committee of the American Academy of Pediatrics stated that up to 3 weeks or less of doxycycline is safe in children of all ages. Our goal was to examine trends in doxycycline treatment for children with Lyme disease.

Methods: We assembled a prospective cohort of children aged 1 to 21 years with Lyme disease who presented to one of eight participating Pedi Lyme Net centers between 2015 and 2023.

Approach to the Patient with Achondroplasia - New Considerations for Diagnosis, Management and Treatment.

Achondroplasia is the most common disproportionate short-stature skeletal dysplasia. Features associated with achondroplasia are rhizomelia, macrocephaly, midface hypoplasia, and typical cognition. Potential medical complications include foramen magnum stenosis, hydrocephalus, middle ear dysfunction, obstructive and central sleep apnea, spinal stenosis and genu varum.

Outcomes and complications of vertebral body tethering by patient gender.

Purpose: Non-fusion surgical options for pediatric scoliosis management such as vertebral body tethering (VBT) offer an alternative to spinal fusion. With this study, we aim to evaluate the postoperative outcomes in boys versus girls who have undergone VBT. Our hypothesis is that girls and boys will have similar outcomes by 2-year follow-up.

Technical considerations of cardiac computed tomography in children.

Cardiac computed tomography angiography (CTA) is a valuable tool in the assessment of congenital and acquired cardiac disease in children. The goal of cardiac CTA is to produce images that are free of motion and provide sufficient characterization of the anatomy in question. Given the complexity of pediatric patient characteristics, including patient size, heart rate, breath-holding capability, and variant anatomy, cardiac CTA technique must be individualized to the patient as well as the indication to answer the clinical question while also minimizing radiation exposure.

DNA-binding affinity and specificity determine the phenotypic diversity in BCL11B-related disorders.

BCL11B is a Cys2-His2 zinc-finger (C2H2-ZnF) domain-containing, DNA-binding, transcription factor with established roles in the development of various organs and tissues, primarily the immune and nervous systems. BCL11B germline variants have been associated with a variety of developmental syndromes. However, genotype-phenotype correlations along with pathophysiologic mechanisms of selected variants mostly remain elusive.

Identifying Quality Improvement Targets After Pediatric Gastrostomy Tube Insertion: A NSQIP-Pediatric Pilot Study.

Background: Gastrostomy tube (GT) placement is one of the most common procedures performed by pediatric surgeons; however, no current national clinical data registry exists to assess GT-specific care processes and morbidity. The American College of Surgeons (ACS) National Surgical Quality Improvement Program-Pediatric (NSQIPPed) GT Pilot was created to provide participants with these data. This study aims to analyze these data to identify variability in perioperative practices and post-operative morbidity in pediatric GT operations and to provide targets for future quality improvement (QI) interventions.

Infant With Beckwith-Wiedemann Requiring Transplant for Hepatic Mesenchymal Hamartoma.

Background: Liver transplantation for unresectable, benign hepatic lesions is rare. Hepatic mesenchymal hamartomas (HMH) are benign, cystic tumors that arise mostly in pediatric populations and can cause compressive symptoms. HMH is rarely associated with placental mesenchymal dysplasia (PMD) and Beckwith-Wiedemann syndrome (BWS).

Single unit-derived connectivity networks in tuberous sclerosis complex reveal propensity for network hypersynchrony driven by tuber-tuber interactions.

Network hypersynchrony is emerging as an important system-level mechanism underlying seizures, as well as cognitive and behavioural impairments, in children with structural brain abnormalities. We investigated patterns of single neuron action potential behaviour in 206 neurons recorded from tubers, transmantle tails of tubers and normal looking cortex in 3 children with tuberous sclerosis. The patterns of neuronal firing on a neuron-by-neuron (autocorrelation) basis did not reveal any differences as a function of anatomy.

Factors Associated with Pediatric Drowning-Associated Lung Injury.

Objective: To identify risk factors for clinically-important drowning-associated lung injury (ciDALI) in children.

Study Design: This was a cross-sectional study of children (0 through 18 years) who presented to 32 pediatric emergency departments (EDs) from 2010 through 2017. We reviewed demographics, comorbidities, prehospital data, chest radiographs reports, and ED course from emergency medical services, medical, and fatality records.

Virtual Support for Bereaved Parents: Acceptability, Feasibility, and Preliminary Efficacy of HOPE Group.

Hospital-based supports for families following the death of a child are rare. Virtual interventions may address key barriers to providing bereavement care, but little is known about their acceptability, feasibility, and efficacy. Our hospital's palliative care program offered a six-week closed virtual support group for bereaved parents five times between 2021 and 2024.

Real-world effectiveness and causal mediation study of BNT162b2 on long COVID risks in children and adolescents.

Background: The impact of pre-infection vaccination on the risk of long COVID remains unclear in the pediatric population. We aim to assess the effectiveness of BNT162b2 on long COVID risks with various strains of the SARS-CoV-2 virus in children and adolescents, using comparative effectiveness methods. We further explore if such pre-infection vaccination can mitigate the risk of long COVID beyond its established protective benefits against SARS-CoV-2 infection using causal mediation analysis.

Topical review: The role of psychologists in healthcare transition.

Objective: Adolescents and young adults (AYAs) with chronic medical conditions often face significant challenges while transitioning from pediatric to adult healthcare (healthcare transition, HCT). Although some guidelines exist on how to facilitate this process, the specific role of psychologists in promoting successful HCT is not clearly defined. In this topical review, we propose a conceptual framework integrating the role of psychology within HCT.

Testosterone Effects on Short-Term Physical, Hormonal, and Neurodevelopmental Outcomes in Infants with 47,XXY/Klinefelter Syndrome: The TESTO Randomized Controlled Trial.

Context: 47,XXY/Klinefelter syndrome (XXY) is associated with impaired testicular function and differences in physical growth, metabolism, and neurodevelopment. Clinical features of XXY may be attributable to inadequate testosterone during the mini-puberty period of infancy.

Objective: We tested the hypothesis that exogenous testosterone treatment positively effects short-term physical, hormonal, and neurodevelopmental outcomes in infants with XXY.

Review: Systematic review and meta-analysis - financial incentives increase engagement with parenting programs for disruptive behavior problems.

Background: To evaluate the effect of financial incentives on engagement in parenting programs for disruptive behavior disorders, as well as effect on child behavior. As a secondary aim, demographic differences were investigated as effect modifiers.

Methods: We searched PubMed, CINAHL, Sociological Abstracts, Cochrane Trials, and PsycINFO for randomized controlled trials and quasi experimental studies offering parents a financial incentive for engagement with parenting programs targeting disruptive behavior in children aged under 18, vs no incentive.

Intercostal Nerve Cryoablation as an Effective Pain Management Strategy in the Nuss Procedure: Reducing Opioid Use and Hospital Stay.

Introduction:  Erector spinae plane (ESP) blocks, thoracic epidural, and patient-controlled analgesia (PCA) have all been used for perioperative pain management in the Nuss procedure without a consensus on what modality produces the best outcomes. Intercostal nerve cryoablation (INC) is a relatively new modality that involves freezing the nerves to prevent pain during recovery. Our hypothesis is that using INC for the Nuss procedure will decrease opioid use, pain scores, and length of stay (LOS) but will increase cost compared with ESP block, thoracic epidural, and PCA.

Towards standardization of person-reported outcomes (PROs) in pediatric diabetes research: A consensus report.

Background: Diabetes ranks among the most common chronic conditions in childhood and adolescence. It is unique among chronic conditions, in that clinical outcomes are intimately tied to how the child or adolescent living with diabetes and their parents or carers react to and implement good clinical practice guidance. It is widely recognized that the individual's perspective about the impact of trying to manage the disease together with the burden of self-management should be addressed to achieve optimal health outcomes.