Mode of Delivery and Subsequent Motor Function in Children With Myelomeningocele Without In Utero Repair.

Objective: To assess the association between mode of delivery and 2-year motor function in children with prenatal diagnosis of myelomeningocele.

Methods: A multisite retrospective cohort study of children with myelomeningocele across 14 NAFTNet (North American Fetal Therapy Network) centers born between 2007 and 2020 who had a physical examination available at 2 years of life. Exclusion criteria were in utero myelomeningocele repair, postnatal myelomeningocele diagnosis, missing data on fetal presentation at delivery, and contraindications to labor.

Pediatric Drug-Associated Pancreatitis Reveals Concomitant Risk Factors and Poor Reliability of Causality Scoring: Report From INSPPIRE.

Objectives: Drug-associated acute pancreatitis (DAP) studies typically focus on single acute pancreatitis (AP) cases. We aimed to analyze the (1) characteristics, (2) co-risk factors, and (3) reliability of the Naranjo scoring system for DAP using INSPPIRE-2 (the INternational Study group of Pediatric Pancreatitis: In search for a cuRE-2) cohort study of acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) in children.

Methods: Data were obtained from ARP group with ≥1 episode of DAP and CP group with medication exposure ± DAP.

The children's brain tumor network (CBTN) - Accelerating research in pediatric central nervous system tumors through collaboration and open science.

Pediatric brain tumors are the leading cause of cancer-related death in children in the United States and contribute a disproportionate number of potential years of life lost compared to adult cancers. Moreover, survivors frequently suffer long-term side effects, including secondary cancers. The Children's Brain Tumor Network (CBTN) is a multi-institutional international clinical research consortium created to advance therapeutic development through the collection and rapid distribution of biospecimens and data via open-science research platforms for real-time access and use by the global research community.

Analysis of INSPPIRE-2 Cohort: Risk Factors and Disease Burden in Children With Acute Recurrent or Chronic Pancreatitis.

Objectives: The objective of this study is to investigate risk factors and disease burden in pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP).

Methods: Data were obtained from INternational Study group of Pediatric Pancreatitis: In search for a cuRE-2 (INSPPIRE-2), the largest multi-center prospective cohort study in pediatric patients with ARP or CP.

Results: Of 689 children, 365 had ARP (53%), 324 had CP (47%).

"Helping the Helpers"-Making Access to Buprenorphine the New Standard of Care for Physicians in State Monitoring Programs.

This commentary argues that the Federation of State Medical Board's (FSMB) recommendations concerning the use of buprenorphine for physicians in their state-affiliated monitoring programs falls short of effectively permitting an evidence-based treatment for opioid use disorder. Although the FSMB acknowledges the benefits of medications for opioid use disorder and recommends that physicians who elect to start on buprenorphine receive treatment safely and privately, the FSMB is opposed to health care professionals practicing while on buprenorphine. Their rationale is based on the notion that physicians are exceptional in their ability to remain in recovery without medications for opioid use disorder and the unsupported assumption that buprenorphine causes significant cognitive impairment.

The Crooked Nose: A Practical Guide to Successful Management.

Learning Objectives: After studying this article, the participant should be able to: (1) Preoperatively evaluate the patient with a crooked nose. (2) Develop a comprehensive preoperative plan specific to the patient. (3) Effectively "deconstruct" and rebuild the crooked nose to address both aesthetic and functional concerns.

Empowering Women as Leaders in Pediatric Anesthesiology: Methodology, Lessons, and Early Outcomes of a National Initiative.

Research has shown that women have leadership ability equal to or better than that of their male counterparts, yet proportionally fewer women than men achieve leadership positions and promotion in medicine. The Women's Empowerment and Leadership Initiative (WELI) was founded within the Society for Pediatric Anesthesia (SPA) in 2018 as a multidimensional program to help address the significant career development, leadership, and promotion gender gap between men and women in anesthesiology. Herein, we describe WELI's development and implementation with an early assessment of effectiveness at 2 years.

Biliary-Atresia-Associated Mannosidase-1-Alpha-2 Gene Regulates Biliary and Ciliary Morphogenesis and Laterality.

Background/aims: Infectious and genetic factors are invoked, respectively in isolated biliary atresia (BA), or syndromic BA, with major extrahepatic anomalies. However, isolated BA is also associated with minor extrahepatic gut and cardiovascular anomalies and multiple susceptibility genes, suggesting common origins.

Methods: We investigated novel susceptibility genes with genome-wide association, targeted sequencing and tissue staining in BA requiring liver transplantation, independent of BA subtype.

The Protective Effects of Calcineurin on Pancreatitis in Mice Depend on the Cellular Source.

Background & Aims: Calcineurin is a ubiquitously expressed central Ca-responsive signaling molecule that mediates acute pancreatitis, but little is known about its effects. We compared the effects of calcineurin expression by hematopoietic cells vs pancreas in mouse models of pancreatitis and pancreatitis-associated lung inflammation.

Methods: We performed studies with mice with hematopoietic-specific or pancreas-specific deletion of protein phosphatase 3, regulatory subunit B, alpha isoform (PPP3R1, also called CNB1), in mice with deletion of CNB1 (Cnb1) and in the corresponding controls for each deletion of CNB1.

Point-of-Care Ultrasound Diagnosis of Perforated Appendicitis in the Pediatric Emergency Department: A Case Series.

Point-of-care ultrasound (POCUS) is being used for clinical decision making with increasing frequency across a broad range of indications in pediatric emergency medicine (PEM). We present a series of 4 patients in whom POCUS was used to facilitate a diagnosis of perforated appendicitis.

The Fate of Homograft Versus Polytetrafluoroethylene Conduits After Neonatal Truncus Arteriosus Repair.

Background: Despite significant improvement in outcomes with truncus arteriosus (TA) repair, right ventricular outflow tract (RVOT) reconstruction with a right ventricular to pulmonary artery (RV-to-PA) conduit remains a source of long-term reintervention and reoperation. This study evaluated our experience with reintervention in homograft and polytetrafluoroethylene (PTFE) RV-to-PA conduits in neonates.

Methods: Primary TA repairs from 2004 to 2016 at a single institution were included.

Focused Cardiac Ultrasound in Pediatric Pulmonary Hypertension.

We present 2 cases of pediatric pulmonary hypertension presenting with respiratory distress. Focused cardiac ultrasound revealed findings consistent with right ventricular dilatation and elevated right ventricular pressure. These findings, in conjunction with the clinical presentation, allowed for early identification and rapid evaluation of a pathologic process that can often go unidentified.

Improvements in intestine transplantation.

Transplantation of the intestine in children has presented significant challenges even as it has become a standard to treat nutritional failure due to short gut syndrome. These challenges have been addressed in part by significant improvements in short and long-term care. Noteworthy enhancements include reduced need for intestine transplantation, drug-sparing immunosuppressive regimens, immune monitoring, and improved surveillance and management of PTLD and non-adherence.

Pharmacokinetics of glycerol phenylbutyrate in pediatric patients 2 months to 2 years of age with urea cycle disorders.

Introduction: Glycerol phenylbutyrate (GPB) is approved in the US and EU for the chronic management of patients ≥2 months of age with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone. GPB is a pre-prodrug, hydrolyzed by lipases to phenylbutyric acid (PBA) that upon absorption is beta-oxidized to the active nitrogen scavenger phenylacetic acid (PAA), which is conjugated to glutamine (PAGN) and excreted as urinary PAGN (UPAGN). Pharmacokinetics (PK) of GPB were examined to see if hydrolysis is impaired in very young patients who may lack lipase activity.

Transient High Pressure in Pancreatic Ducts Promotes Inflammation and Alters Tight Junctions via Calcineurin Signaling in Mice.

Background & Aims: Pancreatitis after endoscopic retrograde cholangiopancreatography (PEP) is thought to be provoked by pancreatic ductal hypertension, via unknown mechanisms. We investigated the effects of hydrostatic pressures on the development of pancreatitis in mice.

Methods: We performed studies with Swiss Webster mice, B6129 mice (controls), and B6129 mice with disruption of the protein phosphatase 3, catalytic subunit, βisoform gene (Cnab mice).

Contribution of Breastfeeding to False-Positive Saliva Polymerase Chain Reaction for Newborn Congenital Cytomegalovirus Screening.

Real-time polymerase chain reaction (PCR) of saliva is highly sensitive for newborn congenital cytomegalovirus (CMV) screening. This study uses nationally published CMV seroprevalence and breastfeeding rates to estimate the contribution of CMV DNA in breast milk to false-positive saliva PCR results. The false-positive rates adjusted for breastfeeding ranged from 0.

Paediatric traumatic brain injury: prognostic insights and outlooks.

Purpose Of Review: Traumatic brain injury (TBI) is a leading cause of death and disability in children. Prognostication of outcome following TBI is challenging in this population and likely requires complex, multimodal models to achieve clinically relevant accuracy. This review highlights injury characteristics, physiological indicators, biomarkers and neuromonitoring modalities predictive of outcome that may be integrated for future development of sensitive and specific prognostic models.

Attitudes and Beliefs Pertaining to Sexual and Reproductive Health Among Unmarried, Female Bhutanese Refugee Youth in Philadelphia.

Purpose: We explored attitudes and beliefs pertaining to sexual and reproductive health (SRH) among unmarried, female, resettled Bhutanese refugees 16-20 years.

Methods: Fourteen interviews were analyzed using the constant comparison method, and major themes were identified.

Results: SRH was stigmatized for unmarried youth, making seeking information about SRH or accessing family planning difficult.

Anterior Segment Optical Coherence Tomography of Previously Operated Extraocular Muscles.

Background And Purpose: To assess the possibility of determining the insertion distance from the limbus of previously operated extraocular rectus muscles (EOM) with the Heidelberg Spectralis anterior segment optical coherence tomography (AS-OCT).

Patient And Method: Subjects with a history of previous strabismus surgery underwent AS-OCT of the EOM before planned additional strabismus surgery. The EOM insertion distances from the limbus were measured pre-operatively on the AS-OCT and compared to the caliper distance measured during the strabismus surgery.

An Intensive, Simulation-Based Communication Course for Pediatric Critical Care Medicine Fellows.

Objective: Effective communication among providers, families, and patients is essential in critical care but is often inadequate in the PICU. To address the lack of communication education pediatric critical care medicine fellows receive, the Children's Hospital of Pittsburgh PICU developed a simulation-based communication course, Pediatric Critical Care Communication course. Pediatric critical care medicine trainees have limited prior training in communication and will have increased confidence in their communication skills after participating in the Pediatric Critical Care Communication course.

Brain-Specific Serum Biomarkers Predict Neurological Morbidity in Diagnostically Diverse Pediatric Intensive Care Unit Patients.

Background: Unexpected neurological morbidity in Pediatric Intensive Care Units (PICUs) remains high and is difficult to detect proactively. Brain-specific biomarkers represent a novel approach for early detection of neurological injury. We sought to determine whether serum concentrations of neuron-specific enolase (NSE), myelin basic protein (MBP), and S100B, specific for neurons, oligodendrocytes, and glia, respectively, were predictive of neurological morbidity in critically ill children.

Evidence-Based Medicine Curriculum Improves Pediatric Emergency Fellows' Scores on In-Training Examinations.

Objectives: The aim of this study was to determine if implementation of our evidence-based medicine (EBM) curriculum had an effect on pediatric emergency medicine fellows' scores on the relevant section of the in-training examination (ITE).

Methods: We obtained deidentified subscores for 22 fellows over 6 academic years for the Core Knowledge in Scholarly Activities (SA) and, as a balance measure, Emergencies Treated Medically sections. We divided the subscores into the following 3 instruction periods: "baseline" for academic years before our current EBM curriculum, "transition" for academic years with use of a research method curriculum with some overlapping EBM content, and "EBM" for academic years with our current EBM curriculum.

Trend and Outcomes of Video Laryngoscope Use Across PICUs.

Objective: Video (indirect) laryngoscopy is used as a primary tracheal intubation device for difficult airways in emergency departments and in adult ICUs. The use and outcomes of video laryngoscopy compared with direct laryngoscopy has not been quantified in PICUs or cardiac ICUs.

Design: Retrospective review of prospectively collected observational data from a multicenter tracheal intubation database (National Emergency Airway Registry for Children) from July 2010 to June 2015.

Practice Variation and Effects of E-mail-only Performance Feedback on Resource Use in the Emergency Department.

Objectives: Higher resource utilization in the management of pediatric patients with undifferentiated vomiting and/or diarrhea does not correlate consistently with improved outcomes or quality of care. Performance feedback has been shown to change physician practice behavior and may be a mechanism to minimize practice variation. We aimed to evaluate the effects of e-mail-only, provider-level performance feedback on the ordering and admission practice variation of pediatric emergency physicians for patients presenting with undifferentiated vomiting and/or diarrhea.