Sedation Management for Critically Ill Children with Pre-Existing Cognitive Impairment.

Objective: To compare current analgesia and sedation management practices between critically ill children with pre-existing cognitive impairment and critically ill neurotypical children, including possible indicators of therapeutic efficacy.

Study Design: This study used secondary analysis of prospective data from the RESTORE clinical trial, with 2449 children admitted to the pediatric intensive care unit and receiving mechanical ventilation for acute respiratory failure. Subjects with a baseline Pediatric Cerebral Performance Category ≥3 were defined as subjects with cognitive impairment, and differences between groups were explored using regression methods accounting for pediatric intensive care unit as a cluster variable.

Patient-Specific Multiscale Modeling of the Assisted Bidirectional Glenn.

Background: First-stage palliation of neonates with single-ventricle physiology is associated with poor outcomes and challenging clinical management. Prior computational modeling and in vitro experiments introduced the assisted bidirectional Glenn (ABG), which increased pulmonary flow and oxygenation over the bidirectional Glenn (BDG) and the systemic-to-pulmonary shunt in idealized models. In this study, we demonstrate that the ABG achieves similar performance in patient-specific models and assess the influence of varying shunt geometry.

Anomalous origin of both the right and left coronary arteries: a singular anomaly†.

Herein, the case of an 8-year-old male child diagnosed with an extremely rare anatomical presentation of a single origin of both the left and right coronary arteries from the non-coronary sinus is presented. The surgical management is also described.

A novel, data-driven conceptualization for critical left heart obstruction.

Background: Qualitative features of aortic and mitral valvar pathology have traditionally been used to classify congenital cardiac anomalies for which the left heart structures are unable to sustain adequate systemic cardiac output. We aimed to determine if novel groups of patients with greater clinical relevance could be defined within this population of patients with critical left heart obstruction (CLHO) using a data-driven approach based on both qualitative and quantitative echocardiographic measures.

Methods: An independent standardized review of recordings from pre-intervention transthoracic echocardiograms for 651 neonates with CLHO was performed.

Predictors of patency for arteriovenous fistulae and grafts in pediatric hemodialysis patients.

Background: Hemodialysis (HD) guidelines recommend permanent vascular access (PVA) in children unlikely to receive kidney transplant within 1 year of starting HD. We aimed to determine predictors of primary and secondary patency of PVA in pediatric HD patients.

Methods: Retrospective chart reviews were performed for first PVAs in 20 participating centers.

Mechanical and structural analysis of the pulmonary valve in congenital heart defects: A presentation of two case studies.

Objective: Congenital Heart Disease (CHD) is the leading cause of pediatric mortality, with many cases affecting the right ventricular outflow tract (RVOT) or pulmonary valve (PV). Understanding the mechanics of the disease condition can provide insight into development of durable repair techniques and bioengineered replacement devices. This work presents a mechanical and structural analysis of the pulmonary valve of two pediatric cases.

Reduced-toxicity allogeneic hematopoietic stem cell transplantation in congenital sideroblastic anemia.

The case of an infant girl with severe congenital sideroblastic anemia associated with a novel molecular defect in mitochondrial transporter SLC25A38 is presented. Her transfusion dependence was fully reversed following allogeneic hematopoietic stem cell transplantation using a modified reduced-intensity conditioning regimen, and she remains healthy 5 years posttransplant.

Health-Related Quality of Life after Pediatric Severe Sepsis.

Background: Pediatric severe sepsis is a public health problem with significant morbidities in those who survive. In this article, we aim to present an overview of the important studies highlighting the limited data available pertaining to long-term outcomes of survivors of pediatric severe sepsis.

Materials And Methods: A review of literature available was conducted using PUBMED/Medline on pediatric severe sepsis outcomes.

An Initiative to Decrease Time to Antibiotics for Patients With Fever and Neutropenia.

The objective was to decrease the time to antibiotic administration for patients arriving in the pediatric emergency department with fever and neutropenia. A multidisciplinary team was assembled and engaged in process analysis through interviews and data review. These findings were used to develop key drivers, and Pareto charts were utilized to prioritize interventions.

The present and future treatment of pediatric type 2 diabetes.

Introduction: Treatment of type 2 diabetes (T2D) in children and adolescents is particularly challenging. Metformin monotherapy is the standard initial treatment for youth with T2D, once metabolic control is restored with insulin in patients who present with ketosis and/or marked hyperglycemia. Insulin, the only other drug approved for use in youth with T2D, is also used as add-on therapy when patients fail metformin mono-therapy.

Vancomycin Monotherapy May Be Insufficient to Treat Methicillin-resistant Staphylococcus aureus Coinfection in Children With Influenza-related Critical Illness.

Background: Coinfection with influenza virus and methicillin-resistant Staphylococcus aureus (MRSA) causes life-threatening necrotizing pneumonia in children. Sporadic incidence precludes evaluation of antimicrobial efficacy. We assessed the clinical characteristics and outcomes of critically ill children with influenza-MRSA pneumonia and evaluated antibiotic use.

Synthetic drug intoxication in children: recognition and management in the emergency department.

When children and adolescents present to the emergency department with agitation or mental status changes, intoxication from synthetic drug use should be in the differential diagnosis. Identifying the responsible compound(s) may be difficult, so asking the patient broad questions and utilizing appropriate diagnostic studies, when indicated, will aid in making the diagnosis and help identify more-serious complications. This issue discusses the challenges presented by the changing chemical formulations of synthetic cannabinoids, cathinones, and phenethylamines; outlines common presentations of intoxication from these substances; and summarizes best practices for evaluating and managing patients who present with intoxication after consumption of these synthetic drugs of abuse.

Whole Exome Sequencing Reveals a Monogenic Cause of Disease in ≈43% of 35 Families With Midaortic Syndrome.

Midaortic syndrome (MAS) is a rare cause of severe childhood hypertension characterized by narrowing of the abdominal aorta in children and is associated with extensive vascular disease. It may occur as part of a genetic syndrome, such as neurofibromatosis, or as consequence of a pathological inflammatory disease. However, most cases are considered idiopathic.

Tourniquet usage in prehospital care and resuscitation of pediatric trauma patients-Pediatric Trauma Society position statement.

Background: Recent mass casualty events in the United States have highlighted the need for public preparedness to prevent death from uncontrolled hemorrhage. The Pediatric Trauma Society (PTS) reviewed the literature regarding pediatric tourniquet usage with the aim to provide recommendations about the utility of this adjunct for hemorrhage control in children.

Methods: Search terms "pediatric" and "tourniquet" were used to query the US National Library of Medicine National Institutes of Health for pertinent literature.

When Clinicians Are Summoned to Testify in Court: Orientation to the Process and Suggestions on Preparation.

There are occasions when clinicians are summoned to court to testify. The reasons for this are wide-ranging. It can be about the provision of patient care that is relevant to a criminal or civil legal matter, or to a malpractice complaint, concerns of safety for a child, child custody issue, allegation of sexual or physical abuse, or being called to testify as an expert witness in your field of expertise.

Outcome of Preterm Infants with Transient Cystic Periventricular Leukomalacia on Serial Cranial Imaging Up to Term Equivalent Age.

Objective: To determine the outcome of preterm infants whose cystic periventricular leukomalacia "disappeared" on serial screening cranial imaging studies.

Study Design: Infants ≤26 weeks of gestation born between 2002 and 2012 who had cranial imaging studies at least twice, the most abnormal study at <28 days of age and another closest to 36 weeks, were reviewed. The outcome of late death (after 36 weeks postmenstrual age) or neurodevelopmental impairment (NDI) in surviving infants at 18-26 months corrected age was compared between the infants with no cystic periventricular leukomalacia on both studies and cystic periventricular leukomalacia that disappeared (cystic periventricular leukomalacia at <28 days but not at 36 weeks), persisted (cystic periventricular leukomalacia on both studies), or appeared late (cystic periventricular leukomalacia only at 36 weeks).

Education for ECMO providers: Using education science to bridge the gap between clinical and educational expertise.

A well-organized educational curriculum for the training of both novice and experienced ECMO providers is critical for the continued function of an institutional ECMO program. ELSO provides guidance for the education for ECMO specialists, physicians and staff, which incorporates "traditional" instructor-centered educational methods, such as didactic lectures and technical skill training. Novel research suggests utilization of strategies that align with principles of adult learning to promote active learner involvement and reflection on how the material can be applied to understand existing and new constructs may be more effective.

Surgical tray optimization as a simple means to decrease perioperative costs.

Background: Health care spending in the US remains excessively high. Aside from complicated, large-scale efforts at health care cost reduction, there are still relatively simple ways in which individual hospitals can cut unnecessary costs from everyday operations. Inspired by recent publications, our group sought to decrease the costs associated with surgical instrument processing at a large, multihospital academic center.

Comparison of valvar and right ventricular function following transcatheter and surgical pulmonary valve replacement.

Objective: Trans-catheter (TC) pulmonary valve replacement (PVR) has become common practice for patients with right ventricular outflow tract obstruction (RVOTO) and/or pulmonic insufficiency (PI). Our aim was to compare PVR and right ventricular (RV) function of patients who received TC vs surgical PVR.

Design: Retrospective review of echocardiograms obtained at three time points: before, immediately after PVR, and most recent.