Evaluating Renal Disease in Pediatric-Onset Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Disease Course, Outcomes, and Predictors of Outcome.

Objective: We aimed to study the disease course, outcomes, and predictors of outcome in pediatric-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) affecting the kidneys.

Methods: Patients eligible for this study had a diagnosis of granulomatosis with polyangiitis (GPA), microscopic polyangiitis, or ANCA-positive pauci-immune glomerulonephritis, were 18 years or younger at diagnosis, had renal disease defined by biopsy or dialysis dependence, and had clinical data at diagnosis and at either 12 or 24 months. Ambispective data from A Registry for Children with Vasculitis/Pediatric Vasculitis Initiative Registry was used.

Categorizing Monogenic Epilepsies by Genetic Mechanisms May Predict Efficacy of the Ketogenic Diet.

Background: The ketogenic diet (KD) is an effective treatment for epilepsy. In recent years, studies have shown favorable efficacy of KD in epilepsy from genetic disorders. In this study, we propose an approach to KD in monogenic epilepsy: we evaluate the utility of categorizing genetic variants based on rational associations with the known mechanisms of KD.

Review of robot-assisted laparoscopic surgery in management of infant congenital urology: Advances and limitations in utilization and learning.

As robotic-assisted (RAL) surgery expanded to treat pediatric congenital disease, infant anatomy and physiology posed unique challenges that prompted adaptations to the technology and surgical technique, which are compiled and reviewed in this manuscript. From the beginning, collaboration with anesthesia is critical for a safe, efficient case including placement of an endotracheal tube rather than a laryngeal mask (LMA) and placement of a nasogastric tube and/or rectal tube to relieve distended stomach or bowel, respectively. Furthermore, end-tidal CO (EtCO ) is important for monitoring and predicting the effects of pneumoperitoneum on caridiovascular physiology, incranial pressure, and risk of acidosis and hypercarbia.

Excessive Oxygen Supplementation in the First Day of Mechanical Ventilation Is Associated With Multiple Organ Dysfunction and Death in Critically Ill Children.

Objectives: To determine if greater cumulative exposure to oxygen despite adequate oxygenation over the first 24 hours of mechanical ventilation is associated with multiple organ dysfunction syndrome at 7 days and inhospital mortality in critically ill children.

Design: Retrospective, observational cohort study.

Setting: Two urban, academic PICUs.

Changes in Neurodevelopmental Outcomes From Age 2 to 10 Years for Children Born Extremely Preterm.

Objectives: Evidence-based care of extremely preterm infants (<28 weeks' gestation) depends heavily on research in which a primary outcome is infant neurodevelopmental impairment (NDI), yet it is unclear how well NDI in infancy predicts long-term NDI. In this study, we aim to assess the relationship between 2- and 10-year neurodevelopment using a well-known 2-year definition and a 10-year definition developed by an expert panel.

Methods: Using data from the Extremely Low Gestational Age Newborn Study cohort, we classified 2-year NDI using definitions developed by the National Institute of Child Health and Human Development Neonatal Research Network.

Non-resuscitation fluid in excess of hydration requirements is associated with higher mortality in critically ill children.

Background: Large volumes of non-resuscitation fluids are often administered to critically ill children. We hypothesize that excess maintenance fluid is a significant contributor to non-resuscitation fluid and that non-resuscitation fluid administered beyond hydration requirements is associated with worse clinical outcomes in critically ill children.

Methods: We evaluated all patients admitted to two large urban pediatric intensive care units (PICU) between January 2010-August 2016 and January 2010-August 2018, respectively, who survived and remained in the hospital for at least 3 days following PICU admission.

Taking the Pulse on Pediatric Simulation: A National Survey of Pediatric Residency Programs' Simulation Practices and Challenges.

Objectives: There is abundant literature on simulation use in individual pediatric residency programs but limited overall data on simulation in US pediatric residency programs. This study sought to determine how US pediatric residency programs use simulation for teaching and assessment and the challenges programs face in their use of simulation.

Methods: The Association of Pediatric Program Director's Healthcare Simulation in Pediatrics Learning Community members developed a 15-multipart question survey on the use of simulation in US pediatric residency programs using best practices in survey design.

Neonatal Respiratory Support on Transport.

Respiratory support is frequently required during neonatal transport. This review identifies the various modalities of respiratory support available during neonatal transport and their appropriate clinical uses. The respiratory equipment required during neonatal transport and appropriate safety checks are also reviewed.

Implementing personalized pathways for cancer follow-up care in the United States: Proceedings from an American Cancer Society-American Society of Clinical Oncology summit.

A new approach to cancer follow-up care is necessary to meet the needs of cancer survivors while dealing with increasing volume and provider shortages, knowledge gaps, and costs to both health care systems and patients. An approach that triages patients to personalized follow-up care pathways, depending on the type(s) and level(s) of resources needed for patients' long-term care, is in use in the United Kingdom and other countries and has been shown to meet patients' needs, more efficiently use the health care system, and reduce costs. Recognizing that testing and implementing a similar personalized approach to cancer follow-up care in the United States will require a multipronged strategy, the American Cancer Society and the American Society of Clinical Oncology convened a summit in January 2018 to identify the needed steps to move this work from concept to implementation.

Genetic variants and pathways implicated in a pediatric inflammatory bowel disease cohort.

In the United States, approximately 5% of individuals with inflammatory bowel disease (IBD) are younger than 20 years old. Studies of pediatric cohorts can provide unique insights into genetic architecture of IBD, which includes Crohn's disease (CD) and ulcerative colitis (UC). Large genome-wide association studies have found more than 200 IBD-associated loci but explain a minority of disease variance for CD and UC.

The Process and Impact of Stakeholder Engagement in Developing a Pediatric Intensive Care Unit Communication and Decision-Making Intervention.

Stakeholder-developed interventions are needed to support pediatric intensive care unit (PICU) communication and decision-making. Few publications delineate methods and outcomes of stakeholder engagement in research. We describe the process and impact of stakeholder engagement on developing a PICU communication and decision-making support intervention.

The Role of Nursing Professionals in the Management of Patients With High-Risk Neuroblastoma Receiving Dinutuximab Therapy.

Neuroblastoma, an embryonic cancer of the sympathetic nervous system, is the most common extracranial solid tumor in childhood. Dinutuximab (formerly called ch14.18), a monoclonal antibody targeting the disialoganglioside GD2, has been shown to significantly improve survival rates in patients with high-risk neuroblastoma.

Population-Based Outcomes Data for Counseling at the Margin of Gestational Viability.

Objective: To survey neonatologists as to how many use population-based outcomes data to counsel families before and after the birth of 22- to 25-week preterm infants.

Study Design: An anonymous online survey was distributed to 1022 neonatologists in the US. Questions addressed the use of population-based outcome data in prenatal and postnatal counseling.

A Multibiomarker-Based Model for Estimating the Risk of Septic Acute Kidney Injury.

Objective: The development of acute kidney injury in patients with sepsis is associated with worse outcomes. Identifying those at risk for septic acute kidney injury could help to inform clinical decision making. We derived and tested a multibiomarker-based model to estimate the risk of septic acute kidney injury in children with septic shock.

Accelerated aging among cancer survivors: from pediatrics to geriatrics.

There are almost 14-million cancer survivors in the United States and the population is growing. Almost two-thirds of these survivors are age 65 or older. Given this, it is imperative to understand the impact of cancer and its therapies on the aging process.

Outcome of treatment in children with hypodiploid acute lymphoblastic leukemia.

One-hundred thirty-nine patients with acute lymphoblastic leukemia (ALL) and hypodiploidy (fewer than 45 chromosomes) were collected from 10 different national ALL study groups and single institutions. Patients were stratified by modal chromosome number into 4 groups: 24 to 29 (N = 46); 33 to 39 (N = 26); 40 to 43 (N = 13); and 44 (N = 54) chromosomes. Nine patients were Philadelphia chromosome (Ph) positive (4 cases: 44 chromosomes; 5 cases: 40-43 chromosomes) and were not considered further.