Renal Response Outcomes of the EuroLupus and National Institutes of Health Cyclophosphamide Dosing Regimens in Childhood-Onset Proliferative Lupus Nephritis.

Objective: We compared clinical characteristics and renal response in patients with childhood-onset proliferative lupus nephritis (LN) treated with the EuroLupus versus National Institutes of Health (NIH) cyclophosphamide (CYC) regimen.

Methods: A retrospective cohort study was conducted at 11 pediatric centers in North America that reported using both CYC regimens. Data were extracted from the electronic medical record at baseline and 3, 6, and 12 months after treatment initiation with CYC.

Factors Associated With Post-Extubation Stridor in Infants Intubated in the Pediatric ICU.

Background: Post-extubation stridor (PES) is a common problem in the pediatric intensive care unit (PICU) and is associated with extubation failure, longer length of stay, and increased mortality. Infants represent a large proportion of PICU admissions and are at higher risk for PES, making identification and mitigation of factors associated with PES important in this age group.

Research Question: What factors are associated with PES in infants (age less than 1 year) intubated in the PICU?

Study Design & Methods: The primary outcome was PES as defined by the need for racemic epinephrine within 6 h of extubation.

Blood Pressure Outcomes in NICU-Admitted Infants with Neonatal Hypertension: A Pediatric Nephrology Research Consortium Study.

Objective: To describe the blood pressure outcomes of infants admitted to the neonatal intensive care unit (NICU) with idiopathic (nonsecondary) hypertension (HTN) who were discharged on antihypertensive therapy.

Study Design: Retrospective, multicenter study of 14 centers within the Pediatric Nephrology Research Consortium. We included all infants with a diagnosis of idiopathic HTN discharged from the NICU on antihypertensive treatment.

Advances in the Cystic Fibrosis Drug Development Pipeline.

Cystic fibrosis is a genetic disease that results in progressive multi-organ manifestations with predominance in the respiratory and gastrointestinal systems. The significant morbidity and mortality seen in the CF population has been the driving force urging the CF research community to further advance treatments to slow disease progression and, in turn, prolong life expectancy. Enormous strides in medical advancements have translated to improvement in quality of life, symptom burden, and survival; however, there is still no cure.

ARFID at 10 years: A Review of Medical, Nutritional and Psychological Evaluation and Management.

Purpose Of Review: Avoidant restrictive food intake disorder (ARFID) is a diagnostic term that was established 10 years ago to describe those patients with an eating disorder, mostly children and adolescents, who have poor nutrition that is not due to body image or weight concerns. This article reviews the diagnosis and subtypes of ARFID, as well as the medical, nutritional and psychological principles of evaluation and management of the disorder.

Recent Findings: In the past 10 years, clinicians have refined their approaches to managing the two major subtypes of ARFID: (1) those patients with a longer-term restriction in the amount and/or variety of the foods they eat, and (2) those patients with a shorter-term decrease in eating because of fear of aversive consequences such as vomiting, choking, GI symptoms or an allergic reaction to food.

CDK6-mediated endothelial cell cycle acceleration drives arteriovenous malformations in hereditary hemorrhagic telangiectasia.

Increased endothelial cell (EC) proliferation is a hallmark of arteriovenous malformations (AVMs) in hereditary hemorrhagic telangiectasia (HHT). The underlying mechanism and disease relevance of this abnormal cell proliferative state of the ECs remain unknown. Here, we report the identification of a CDK6-driven mechanism of cell cycle progression deregulation directly involved in EC proliferation and HHT vascular pathology.

The prevalence and outcomes of hyponatremia in children with COVID-19 and multisystem inflammatory syndrome in children (MIS-C).

Introduction: To assess the prevalence of hyponatremia among pediatric patients with coronavirus disease 2019 (COVID-19) and Multisystem Inflammatory Syndrome in Children (MIS-C) and determine if pediatric hyponatremia was associated with an increased length of stay, higher rates of mechanical ventilation, and/or elevated inflammatory markers on admission as compared to eunatremic patients.

Methods: Electronic health records were retrospectively analyzed for 168 children less than 18 years old with COVID-19 or MIS-C who were admitted to pediatric units within the Northwell Health system. The primary exposure was hyponatremic status (serum sodium <135 mEq/L) and the primary outcomes were length of stay, mechanical ventilation usage and increased inflammatory markers.

Association and Repair of Right Aortic Arch With Aberrant Left Subclavian Artery With Subclavian Stenosis.

Surgical repair of right aortic arch and aberrant left subclavian artery has traditionally involved ligamentum division. Such patients can have stenosis at the origin of the aberrant subclavian artery either at the time of presentation or later. The more recently popularized repair involving resection of Kommerell diverticulum with transfer of the subclavian artery to the left carotid artery allows resection of the stenotic segment and serves as an effective treatment for subclavian stenosis as well.

Gut microbiome perturbation, antibiotic resistance, and Escherichia coli strain dynamics associated with international travel: a metagenomic analysis.

Background: Culture-based studies have shown that acquisition of extended-spectrum β-lactamase-producing Enterobacterales is common during international travel; however, little is known about the role of the gut microbiome before and during travel, nor about acquisition of other antimicrobial-resistant organisms. We aimed to identify (1) whether the gut microbiome provided colonisation resistance against antimicrobial-resistant organism acquisition, (2) the effect of travel and travel behaviours on the gut microbiome, and (3) the scale and global heterogeneity of antimicrobial-resistant organism acquisition.

Methods: In this metagenomic analysis, participants were recruited at three US travel clinics (Boston, MA; New York, NY; and Salt Lake City, UT) before international travel.

The diversity of hypospadias management in North America: a survey of pediatric urologists.

Purpose: To determine trends in hypospadias management, including surgical techniques and perioperative care, by pediatric urologists in North America.

Methods: An anonymous online survey was devised to assess approaches to hypospadias repair and management, including anesthetic considerations, catheter placement, choice of dressing, and postoperative antibiotic treatment. The survey was sent to all practicing members of the Societies for Pediatric Urology.

Neurodevelopmental and behavioral outcomes of very preterm infants: latent profile analysis in the Environmental influences on Child Health Outcomes (ECHO) Program.

Background: Very preterm infants are at high risk for neurodevelopmental impairments. We used a child-centered approach (latent profile analysis [LPA]) to describe 2-year neurobehavioral profiles for very preterm infants based on cognitive, motor, and behavioral outcomes. We hypothesized that distinct outcome profiles would differ in the severity and co-occurrence of neurodevelopmental and behavioral impairment.

Characterizing Lymphovascular Invasion in Pediatric and Adolescent Malignant Ovarian Nongerminomatous Germ Cell Tumors: A Report from the Children's Oncology Group.

Background: Lymphovascular invasion (LVI) has been identified as a poor prognostic factor for a variety of tumors; however, its significance in malignant ovarian germ cell tumors (MOGCT) in pediatric and adolescent patients is not well described. We aim to clarify the significance of LVI in the subset of patients with nongerminomatous MOGCT.

Methods: Records of patients 0-20 years of age with MOGCT enrolled on Children's Oncology Group study AGCT0132 were reviewed.

ADHD Diagnosis in Children of Non-US-Born Parents: A Cross-Sectional Analysis.

Objective(s): To assess the likelihood of ADHD diagnosis in children of non-US-born caregivers relative to children of US-born caregivers.

Method: Cross-sectional analysis of a combined 2016 to 2019 National Survey of Children's Health dataset ( = 109,881) was performed to identify associations between caregiver's birth outside the U.S.

Diagnostic and Communication Challenges in Cystic Fibrosis Newborn Screening.

As of December 2009, cystic fibrosis (CF) newborn screening (NBS) is performed in all 50 US states and the District of Columbia. Widespread implementation of CF newborn screening (CFNBS) in the US and internationally has brought about new and varied challenges. Immunoreactive trypsinogen (IRT) remains the first, albeit imperfect, biomarker used universally in the screening process.

Treatment of High-Risk Neuroblastoma.

High-risk neuroblastoma is a highly aggressive solid tumor that most commonly presents in early childhood. Advances in treatment through decades of clinical trials and research have led to improved outcomes. This review provides an overview of the current state of treatment for high-risk neuroblastoma.

Unwrapping Nephrogenic Rests and Nephroblastomatosis for Pediatric Surgeons: A Systematic Review Utilizing the PICO Model by the APSA Cancer Committee.

Background: Nephrogenic rests (NR) may represent precursor lesions for Wilms tumor (WT), but their clinical course is not fully understood and no guidelines for treatment exist. This study sought to evaluate the outcomes of pediatric patients with NRs related to traditional chemotherapy and surgery.

Methods: A PRISMA-P-compliant literature search was conducted in MEDLINE, Embase, CINAHL, Web of Science, COCHRANE, and SCOPUS from inception to June 2021.

Breast Milk Production Variability Among Mothers of Preterm Infants.

Mothers of preterm infants are at risk for inadequate milk production. Pumping logs are often used to both encourage lactation in the first week and track its efficacy. Our objectives were to determine whether mothers of preterm infants who keep pumping logs are demographically different from those who do not and to determine whether this practice affects the amount of mother's own milk (MOM) fed to their infants.

Trends and Disparities in Firearm Deaths Among Children.

Background And Objectives: In 2020, firearm injuries became the leading cause of death among US children and adolescents. This study aimed to evaluate new 2021 data on US pediatric firearm deaths and disparities to understand trends compared with previous years.

Methods: Centers for Disease Control and Prevention Wide-ranging Online Data for Epidemiologic Research was queried for firearm mortalities in children/adolescents from 2018 to 2021.