Viral Detection by Reverse Transcriptase Polymerase Chain Reaction in Upper Respiratory Tract and Metagenomic RNA Sequencing in Lower Respiratory Tract in Critically Ill Children With Suspected Lower Respiratory Tract Infection.

Objectives: Viral lower respiratory tract infection (vLRTI) contributes to substantial morbidity and mortality in children. Diagnosis is typically confirmed by reverse transcriptase polymerase chain reaction (RT-PCR) of nasopharyngeal specimens in hospitalized patients; however, it is unknown whether nasopharyngeal detection accurately reflects presence of virus in the lower respiratory tract (LRT). This study evaluates agreement between viral detection from nasopharyngeal specimens by RT-PCR compared with metagenomic next-generation RNA sequencing (RNA-Seq) from tracheal aspirates (TAs).

Health-related quality of life following valoctocogene roxaparvovec gene therapy for severe hemophilia A in the phase 3 trial GENEr8-1.

Background: Severe hemophilia A (HA) negatively impacts health-related quality of life (HRQOL).

Objectives: We aimed to analyze HRQOL in adult men with severe HA without inhibitors after valoctocogene roxaparvovec gene transfer in the phase 3 trial GENEr8-1.

Methods: Participant-reported outcomes were the hemophilia-specific quality of life questionnaire for adults (Haemo-QOL-A), the EQ-5D-5L instrument, the Hemophilia Activities List (HAL), and the Work Productivity and Activity Impairment Questionnaire: Hemophilia Specific (WPAI+CIQ:HS).

Perioperative Neonatal Acute Kidney Injury Is Common: Risk Factors for Poor Outcomes.

Objective: Perioperative acute kidney injury (AKI) is associated with poor patient outcomes. The epidemiology of perioperative AKI is characterized in children and to a lesser extent in neonates with cardiac disease. We hypothesized that the prevalence of noncardiac perioperative AKI in neonates is higher than in older children.

The Risk of Developmental Dysplasia of the Hip in Premature Infants with Breech Presentation at Birth.

Objective: This article evaluates the risk (defined here as incidence per 1,000 infants) of developmental dysplasia of the hip (DDH) in premature infants undergoing hip ultrasound (HUS) for breech presentation only compared with other indications.

Study Design: Retrospective study of infants born between January 1, 2009 and December 31, 2018 at <37 weeks' gestation with a HUS obtained in the first year of life, at Nationwide Children's Hospital, the only available facility for pediatric radiology and orthopaedic services in central Ohio. We calculated risk differences and odds ratios of DDH by the indication of HUS.

Pharmacist involvement in blood culture follow up for patients discharged from the emergency department.

Study Objective: This study evaluates the time to attempted patient contact for positive blood cultures in patients discharged from the Emergency Department (ED) resulting when an Emergency Medicine (EM) pharmacist is on-duty compared to off-duty.

Methods: This single center, retrospective study included patients who were discharged from the ED and had subsequent positive blood cultures. Blood cultures were reviewed utilizing an algorithm previously approved and implemented by an interdisciplinary team in 2016.

Human pulmonary microvascular endothelial cell DDAH1-mediated nitric oxide production promotes pulmonary smooth muscle cell apoptosis in co-culture.

Bronchopulmonary dysplasia (BPD) is the most common chronic lung disease in preterm infants, and pulmonary hypertension (PH) develops in 25%-40% of patients with BPD, increasing morbidity and mortality. BPD-PH is characterized by vasoconstriction and vascular remodeling. Nitric oxide (NO) is a pulmonary vasodilator and apoptotic mediator made in the pulmonary endothelium by NO synthase (eNOS).

Provision and availability of genomic medicine services in Level IV neonatal intensive care units.

Purpose: To describe variation in genomic medicine services across level IV neonatal intensive care units (NICUs) in the United States and Canada.

Methods: We developed and distributed a novel survey to the 43 level IV NICUs belonging to the Children's Hospitals Neonatal Consortium, requesting a single response per site from a clinician with knowledge of the provision of genomic medicine services.

Results: Overall response rate was 74% (32/43).

A ROTEM-guided algorithm aimed to reduce blood product utilization during neonatal and infant cardiac surgery.

Background: Neonates and infants undergoing cardiac surgery tend to receive high volumes of blood products. The use of rotational thromboelastometry (ROTEM) has been shown to reduce the administration of blood products in adults after cardiac surgery. We sought to develop a targeted administration of blood products based on ROTEM to reduce blood product utilization during and after neonatal and infant cardiac surgery.

Body mass index in relation to symptom presentation on diagnosis of eosinophilic esophagitis in children.

Background: The relationship between body mass index (BMI) and eosinophilic esophagitis (EoE) among children is not well characterized.

Objective: To evaluate the presentations of EoE in pediatric patients of different weight classes.

Methods: Records of newly diagnosed children with EoE at an academic center from 2015 to 2018 were evaluated regarding demographics, symptom presentation, and endoscopic findings and compared among the underweight, normal weight, overweight, and obese groups.

Perinatal-lethal nonimmune fetal hydrops attributed to -associated bone marrow failure.

Pathogenic variants in , a gene critical to the self-renewal and proliferation of hematopoietic stem cells, are known to cause a rare bone marrow failure syndrome associated with amegakaryocytic thrombocytopenia and bilateral radioulnar synostosis known as RUSAT2. However, the spectrum of disease seen with causal variants in is broad, ranging from mildly affected adults to fetal loss. We report two cases of infants born preterm who presented at birth with symptoms of bone marrow failure including severe anemia, hydrops, and petechial hemorrhages; radioulnar synostosis was not observed in either patient, and, unfortunately, neither infant survived.

Proceedings of the 10th Pediatric Dermatology Research Alliance (PeDRA) Annual Conference.

The 10th Pediatric Dermatology Research Alliance (PeDRA) Annual Conference occurred November 3-5, 2022 in Bethesda, Maryland. This conference was the first in-person PeDRA conference after 2 years of a virtual format due to COVID-19. Fittingly, given the effects of the pandemic, the conference theme was "Reimagining Community.

Using Billing Codes to Create a Pediatric Functional Status e-Score for Children Receiving Inpatient Rehabilitation.

Objective: Provide proof-of-concept for development of a Pediatric Functional Status eScore (PFSeS). Demonstrate that expert clinicians rank billing codes as relevant to patient functional status and identify the domains that codes inform in a way that reliably matches analytical modeling.

Design: Retrospective chart review, modified Delphi, and nominal group techniques.

Concurrent Choice Assessment of Preference and Substitutability of E-cigarettes and Heated Tobacco Products for Combustible Cigarettes Among African American and White Smokers.

Introduction: Alternative nicotine delivery products, including electronic cigarettes (e-cigarettes) and heated tobacco products (HTPs), contain fewer toxicants than combustible cigarettes and offer a potential for harm reduction. Research on the substitutability of e-cigarettes and HTPs is crucial for understanding their impact on public health. This study examined subjective and behavioral preferences for an e-cigarette and HTP relative to participants' usual brand combustible cigarette (UBC) in African American and White smokers naïve to alternative products.

Two-Year Outcomes of Valoctocogene Roxaparvovec Therapy for Hemophilia A.

Background: Valoctocogene roxaparvovec delivers a B-domain-deleted factor VIII coding sequence with an adeno-associated virus vector to prevent bleeding in persons with severe hemophilia A. The findings of a phase 3 study of the efficacy and safety of valoctocogene roxaparvovec therapy evaluated after 52 weeks in men with severe hemophilia A have been published previously.

Methods: We conducted an open-label, single-group, multicenter, phase 3 trial in which 134 men with severe hemophilia A who were receiving factor VIII prophylaxis received a single infusion of 6×10 vector genomes of valoctocogene roxaparvovec per kilogram of body weight.

The Impact of Restrictive Transfusion Practices on Hemodynamically Stable Critically Ill Children Without Heart Disease: A Secondary Analysis of the Age of Blood in Children in the PICU Trial.

Objectives: Guidelines recommend against RBC transfusion in hemodynamically stable (HDS) children without cardiac disease, if hemoglobin is greater than or equal to 7 g/dL. We sought to assess the clinical and economic impact of compliance with RBC transfusion guidelines.

Design: A nonprespecified secondary analysis of noncardiac, HDS patients in the randomized trial Age of Blood in Children (NCT01977547) in PICUs.

Associations between testing and treatment pathways in lesional temporal or extratemporal epilepsy: A census survey of NAEC center directors.

Objective: The evaluation to determine candidacy and treatment for epilepsy surgery in persons with drug-resistant epilepsy (DRE) is not uniform. Many non-invasive and invasive tests are available to ascertain an appropriate treatment strategy. This study examines expert response to clinical vignettes of magnetic resonance imaging (MRI)-positive lesional focal cortical dysplasia in both temporal and extratemporal epilepsy to identify associations in evaluations and treatment choice.

"Do No Harm": Promoting Anti-Racist Policing in Pediatric Emergency Departments Through 20 Practice Change Considerations.

Children represent some of the most vulnerable and most valuable members of society. When acutely ill or injured, pediatric emergency departments (EDs) provide first line, specialized care for children and adolescents. Unique and unpredictable, the pediatric ED environment requires a wide range of health care professionals to care for children and their families and often includes hospital-employed security professionals and local law enforcement personnel to ensure safe and protective spaces paramount for optimal patient care.