Mortality Patterns in Pediatric Pulmonary Vein Stenosis: Insights Into Right Ventricular Systolic Pressure Associations.

Background: Pulmonary vein stenosis in children is associated with a poor prognosis. However, the cause and risk factors for mortality remain uncertain.

Methods: This retrospective, single-center study identified children with primary and secondary pulmonary vein stenosis through a cardiac catheterization database.

Eighth Annual Society of Thoracic Surgeons Pedimacs Report.

Background: The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), supported by The Society of Thoracic Surgeons, provides detailed information on pediatric patients supported with ventricular assist devices (VADs).

Methods: From September 19, 2012, to December 31, 2023, there were 1648 devices in 1349 patients (<19 years) from 39 North American Hospitals with 100 patients enrolled in 2023.

Results: Cardiomyopathy was the most common underlying etiology (59%), followed by congenital heart disease (26%) and myocarditis (8%).

Potentialities and critical issues of liquid biopsy in clinical practice: An umbrella review.

Background: Liquid biopsy (LB) is a laboratory test performed on a fluid sample aiming at analyzing molecular data derived from circulating cells and related entities, or from nucleic acids. This umbrella review aims to map and evaluate the evidence supporting the use of LB in medicine across different medical specialities and conditions.

Methods: We searched three repositories from database inception up to October 1, 2023 and we included meta-analyses of observational studies reporting data on the use of LB, compared to gold standard, and its accuracy (area under the curve, AUC).

Epidemiology of diagnostic errors in pediatric emergency departments using electronic triggers.

Objectives: We applied three electronic triggers to study frequency and contributory factors of missed opportunities for improving diagnosis (MOIDs) in pediatric emergency departments (EDs): return visits within 10 days resulting in admission (Trigger 1), care escalation within 24 h of ED presentation (Trigger 2), and death within 24 h of ED visit (Trigger 3).

Methods: We created an electronic query and reporting template for the triggers and applied them to electronic health record systems of five pediatric EDs for visits from 2019. Clinician reviewers manually screened identified charts and initially categorized them as "unlikely for MOIDs" or "unable to rule out MOIDs" without a detailed chart review.

Consensus outcomes between health professionals and parents for oral corticosteroids in treating preschool wheeze: a multi-national survey and nominal group technique study.

Objective: To obtain priority consensus for outcome measures of oral corticosteroid treatment of preschool wheeze that represent stakeholder groups.

Design: (1) A systematic review to identify a set of outcome measures; (2) an international survey for healthcare professionals (HCPs) and a nominal group meeting with parents; (3) a final consensus nominal group meeting with key HCPs (trial investigators and paediatric emergency medicine clinicians) and the same parent group.

Main Outcome Measures: Consensus priority of treatment outcome measures, outcome minimal clinically important differences (MCIDs) and level of concerns about adverse effects.

Heart Transplantation Outcomes for Fontan Patients When Using Contemporary Strategies: Heart-Liver Transplantation and Ventricular Assist Device Therapy.

Background: With an increasing number of Fontan patients surviving into adulthood, the burden of end-stage heart failure is increasing. Prior studies have reported suboptimal heart transplantation (HTx) outcomes. Therefore, the authors describe their institutional experience of HTx in patients with Fontan circulation failure, including heart-liver transplantation (HLTx) and pretransplant systemic ventricular assist device (SVAD) therapies.

Oxidative Stress Early After Hematopoietic Stem Cell Transplant.

HSCT conditioning regimens cause massive lysis of hematopoietic cells with release of toxic intracellular molecules into the circulation. To describe the response to oxidative stress early after hemopoietic stem cell transplantation (HSCT) and assess the association of early oxidative stress with later transplant outcomes. Key components of in the body's physiological response to oxidative stress were studied in a cohort of 122 consecutive pediatric allogeneic HSCT recipients.

A Controversial Clinical Case of a Child With Hypoplastic Left Heart Syndrome.

We present the case of a child born with hypoplastic left heart syndrome. After developing multiple complications following the first surgical stage (Norwood procedure), her parents decide not to proceed with the second stage (Glenn operation). Cardiac surgeons, pediatric intensivists, a psychologist, and a bioethicist analyze whether further surgical intervention is ethically obligatory.

Screening for Structural Heart Defects: A Single-Center Retrospective Cost Analysis for Fetal Echocardiography in Adults with Congenital Heart Disease.

Fetal echocardiography (FE) is recommended for parents with congenital heart disease (pCHD) due to a 3-6% recurrence risk of congenital heart disease (CHD). This study aimed to evaluate the cost of FE for detecting neonatal CHD in pCHD. FE data were collected between 12/2015 and 12/2022.

Assessing Clinical Improvement of Infants Hospitalized for Respiratory Syncytial Virus-Related Critical Illness.

Background: Pediatric respiratory syncytial virus (RSV)-related acute lower respiratory tract infection (LRTI) commonly requires hospitalization. The Clinical Progression Scale Pediatrics (CPS-Ped) measures level of respiratory support and degree of hypoxia across a range of disease severity, but it has not been applied in infants hospitalized with severe RSV-LRTI.

Methods: We analyzed data from a prospective surveillance registry of infants hospitalized for RSV-related complications across 39 U.

The impact of highly effective modulator therapy on sinusitis and dysosmia in young children with cystic fibrosis: a prospective study protocol.

Background: Chronic rhinosinusitis (CRS) and olfactory dysfunction (OD) are prevalent disease complications in people with cystic fibrosis. These understudied comorbidities significantly impact quality of life. The impact of highly effective modulator therapy (HEMT) in young children with cystic fibrosis (YCwCF) on these disease complications is unknown.

Trochleoplasty Utilization in the Management of Patellofemoral Instability: Results From an International Survey of Surgeons.

Background: Considerable variability exists in the described clinical and radiographic indications for use, surgical techniques, postoperative management, and risk profile after trochleoplasty for the management of patellofemoral instability (PFI). In areas of clinical uncertainty, a cohesive summary of expert opinion and identification of areas of variation in current practice can be useful in guiding current practice and future research efforts.

Purpose: To assess the current indications for use, surgical techniques, postoperative rehabilitation practices, and observed complication profile for trochleoplasty in the management of PFI among surgeons who perform this procedure.

Community Implementation and Evaluation of the Chicago Parent Program.

The purpose of this study was to describe and evaluate the implementation of the Chicago Parent Program (CPP) in a community setting. Included are details of the co-designed implementation by academic and community partners, and parenting and child behavior outcomes. Two hundred eighty caregivers of children aged 2-5 years participated in the CPP in seven community sites.

Communicating neurological prognosis in the prenatal period: a narrative review and practice guidelines.

Clinicians may face an array of challenges in conducting fetal neurological consultations including prognostic uncertainty, a lack of training in fetal counseling, and limited opportunity to build rapport with families. In this setting, it is critical to employ high-quality, family-centered care to allow expectant parents to make informed decisions. Despite the challenges and gravity of these consultations, there remains limited data outlining best conduct and communication practices.

Rhabdomyosarcoma Surgical Update.

Rhabdomyosarcoma (RMS) tumors arise from mesenchymal tissue and represent half of pediatric sarcomas, which in turn make up 7% of pediatric tumors. Advances in local control therapy of RMS have improved outcomes after surgical resection of the primary tumor, either before or after induction chemotherapy, even in the setting of metastatic disease. The utilization of diagnostic core needle and sentinel node biopsy techniques for lymph node staging are becoming more widely used.

A thermally polarized, dissolved-phase Xe phantom for quality-control and multisite comparisons of gas-exchange imaging.

Harmonizing and validating Xe gas exchange imaging across multiple sites is hampered by a lack of a quantitative standard that 1) displays the unique spectral properties of Xe observed from human subjects in vivo and 2) has short enough T times to enable practical imaging. This work describes and demonstrates the development of two dissolved-phase, thermally polarized phantoms that mimic the in-vivo, red blood cell and membrane resonances of Xe dissolved in human lungs. Following optimization, combinations of two common organic solvents, acetone and dimethyl sulfoxide, resulted in two in-vivo-like dissolved-phase Xe phantoms yielding chemical shifts of 212.

Formulation of Quality Improvement Methodology for Risk Mitigation in Congenital Cardiac Catheterization.

The C3PO collaborative, with a history of successful quality improvement (QI) initiatives, leveraged registry participants to develop a multi-center QI initiative to reduce adverse events (AEs) in congenital cardiac catheterization. A 32-person, interdisciplinary working group analyzed audited data for all congenital cardiac catheterization cases from 2014-2017. The primary outcome was the occurrence of any high-severity (level 3/4/5) AE.

Prevalence of metal hypersensitivity in pediatric spine surgery.

Purpose: Delayed metal hypersensitivity reactions can cause complications in spine surgery. Currently, there is no information on the prevalence of metal hypersensitivity in pediatric patients undergoing spine surgery. The objective of this study is to determine the prevalence of metal hypersensitivity in pediatric patients undergoing spinal instrumentation.

The role of CNBP in brain atrophy and its targeting in myotonic dystrophy type 2.

Myotonic Dystrophy type 2 (DM2) is a multisystem disease affecting many tissues, including skeletal muscle, heart, and brain. DM2 is caused by unstable expansion of CCTG repeats in an intron 1 of a gene coding for cellular nuclear binding protein (CNBP). The expanded CCTG repeats cause DM2 pathology due to the accumulation of RNA CCUG repeats, which affect RNA processing in patients' cells.

Implementing Patient Protection Radiation Dose Alerts for Pediatric Cardiac Catheterization Examinations.

Background: Advancements in cardiac catheterization have improved survival for pediatric congenital heart disease patients, but the associated ionizing radiation risks necessitate ethical consideration.

Methods: This study presents an empirical model, developed from 3131 unique pediatric procedures, to establish alert levels based on a patient's lateral thickness of the thorax for various procedural categories during diagnostic or interventional cardiac catheterization. The model uses linear regression of logarithmic reference air kinetic energy released per unit mass (KERMA) and air KERMA area product, also referred to as dose area product, to set alert levels at the top 95% and 99% of patient data.

Accuracy of pathogen diagnostic codes for acute hematogenous musculoskeletal infections in children.

Administrative databases are powerful tools for pediatric research but lack patient-level microbiology results. This study aimed to determine the accuracy of pathogen discharge diagnosis codes for children hospitalized with acute hematogenous musculoskeletal infections (MSKIs). Medical records for 244 children hospitalized with acute hematogenous MSKIs were manually reviewed to determine which bacterial pathogen, if any, was identified for each MSKI based on microbiology results obtained during the hospitalization.

Engaging Students in a Dialysis Unit: A Pilot Study.

Background: Seeking to provide early paediatric nephrology exposure to medical students in the United States, we implemented the Kids In Dialysis, Nephrology Exposure and Education (KIDNEE) club. This club served as an educational intervention in which preclinical medical students were paired with paediatric dialysis patients, as patient buddies.

Approach: Students were recruited for involvement in the club through the medical school Paediatric Interest Group.

Six-Month Outcomes in the Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children Study.

Importance: Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening complication of COVID-19 infection. Data on midterm outcomes are limited.

Objective: To characterize the frequency and time course of cardiac dysfunction (left ventricular ejection fraction [LVEF] <55%), coronary artery aneurysms (z score ≥2.