Time to Pathogen Detection for Non-ill Versus Ill-Appearing Infants ≤60 Days Old With Bacteremia and Meningitis.

Objectives: We sought to determine the time to pathogen detection in blood and cerebrospinal fluid (CSF) for infants ≤60 days old with bacteremia and/or bacterial meningitis and to explore whether time to pathogen detection differed for non-ill-appearing and ill-appearing infants.

Methods: We included infants ≤60 days old with bacteremia and/or bacterial meningitis evaluated in the emergency departments of 10 children's hospitals between July 1, 2011, and June 30, 2016. The microbiology laboratories at each site were queried to identify infants in whom a bacterial pathogen was isolated from blood and/or CSF.

A pilot investigation of food insecurity among children seen in an outpatient pediatric nephrology clinic.

Food insecurity (FI) is common - affecting one in six American households with children. FI is defined as limited or uncertain availability of nutritionally adequate and safe foods. Awareness of food insecurity and its impact on health has increased since the American Academy of Pediatrics 2015 policy statement, "Promoting Food Security for All Children.

Neonatal hypertension: cases, causes, and clinical approach.

Neonatal hypertension is increasingly recognized as dramatic improvements in neonatal intensive care, advancements in our understanding of neonatal physiology, and implementation of new therapies have led to improved survival of premature infants. A variety of factors appear to be important in determining blood pressure in neonates, including gestational age, birth weight, and postmenstrual age. Normative data on neonatal blood pressure values remain limited.

Participation in Daily Life: Influence on Quality of Life in Ambulatory Children with Cerebral Palsy.

Background: Cerebral palsy (CP) is a chronic condition that affects children and has an impact on social and physical activity, as well as participation in daily life. Participation and quality of life (QOL) are 2 important measures of successful rehabilitation that have not been well studied in children with CP.

Objective: To report levels of participation and examine the relationship of participation to QOL in ambulatory children with CP.

Transsplenic splenoportography and portal venous interventions in pediatric patients.

Background: Data regarding transsplenic portal venous access for diagnostic imaging and endovascular intervention in children are limited, possibly due to concerns regarding high bleeding risks and resultant underutilization.

Objective: To investigate the safety and utility of transsplenic splenoportography and portal venous interventions in children.

Materials And Methods: A retrospective review was performed of all pediatric patients undergoing percutaneous transsplenic portal venous access and intervention at two large tertiary pediatric institutions between January 2012 and April 2017 was performed.

Epinephrine, auto-injectors, and anaphylaxis: Challenges of dose, depth, and device.

Objective: This review was undertaken to review epinephrine dosing, site and route of administration, focusing on special populations (patients weighing less than 15 kg, and obese patients); and to discuss storage and delivery of epinephrine in prehospital and hospital settings.

Data Sources: Review of published literature.

Study Selection: Relevance.

COQ2 nephropathy: a treatable cause of nephrotic syndrome in children.

Background: Nephrotic syndrome can be caused by a subgroup of mitochondrial diseases classified as primary coenzyme Q (CoQ) deficiency. Pathogenic COQ2 variants are a cause of primary CoQ deficiency and present with phenotypes ranging from isolated nephrotic syndrome to fatal multisystem disease.

Case-diagnosis/treatment: We report three pediatric patients with COQ2 variants presenting with nephrotic syndrome.

Recessive mutations in VPS13D cause childhood onset movement disorders.

VPS13 protein family members VPS13A through VPS13C have been associated with various recessive movement disorders. We describe the first disease association of rare recessive VPS13D variants including frameshift, missense, and partial duplication mutations with a novel complex, hyperkinetic neurological disorder. The clinical features include developmental delay, a childhood onset movement disorder (chorea, dystonia, or tremor), and progressive spastic ataxia or paraparesis.

Genomic Amplifications and Distal 6q Loss: Novel Markers for Poor Survival in High-risk Neuroblastoma Patients.

Background: Neuroblastoma is characterized by substantial clinical heterogeneity. Despite intensive treatment, the survival rates of high-risk neuroblastoma patients are still disappointingly low. Somatic chromosomal copy number aberrations have been shown to be associated with patient outcome, particularly in low- and intermediate-risk neuroblastoma patients.

Clinical and Imaging Predictors of Surgical Splenorenal Shunt Dysfunction in Pediatric Patients.

Purpose: Few established criteria exist to prompt angiographic evaluation and intervention for surgically created splenorenal shunts (SRS). Clinical and Doppler ultrasound (DUS) imaging predictors of shunt dysfunction were evaluated in this retrospective study.

Materials And Methods: Consecutive patients undergoing SRS angiography over a 10-year period were retrospectively identified.

Prematurity and future kidney health: the growing risk of chronic kidney disease.

Purpose Of Review: The purpose of this review is to describe the role prematurity plays in the development of chronic kidney disease (CKD) and to discuss potential reasons for this association including decreased nephron mass, as well as postnatal insults such as neonatal acute kidney injury (nAKI).

Recent Findings: New observational studies in humans and experimental studies in animal models have strengthened the association between prematurity, low birth weight and CKD. Growing evidence suggests increased susceptibility to CKD is caused by decreased nephron mass at birth.

Intravenous immunoglobulin with prednisone and risk-adapted chemotherapy for children with opsoclonus myoclonus ataxia syndrome associated with neuroblastoma (ANBL00P3): a randomised, open-label, phase 3 trial.

Purpose: No previous clinical trial has been conducted for patients with neuroblastoma associated opsoclonus myoclonus ataxia syndrome (OMA), and current treatment is based on case reports. To evaluate the OMA response to prednisone and risk-adapted chemotherapy and determine if the addition of intravenous gammaglobulin (IVIG) further improves response, the Children's Oncology Group designed a randomized therapeutic trial.

Patient And Methods: Eligible subjects were randomized to receive twelve cycles of IVIG (IVIG+) or no IVIG (NO-IVIG) in addition to prednisone and neuroblastoma risk-adapted chemotherapy.

A Shared Pattern of β-Catenin Activation in Bronchopulmonary Dysplasia and Idiopathic Pulmonary Fibrosis.

Wnt/β-catenin signaling is necessary for normal lung development, and abnormal Wnt signaling contributes to the pathogenesis of both bronchopulmonary dysplasia (BPD) and idiopathic pulmonary fibrosis (IPF), fibrotic lung diseases that occur during infancy and aging, respectively. Using a library of human normal and diseased human lung samples, we identified a distinct signature of nuclear accumulation of β-catenin phosphorylated at tyrosine 489 and epithelial cell cytosolic localization of β-catenin phosphorylated at tyrosine 654 in early normal lung development and fibrotic lung diseases BPD and IPF. Furthermore, this signature was recapitulated in murine models of BPD and IPF.

Novel pregnancy-triggered episodes of CAPOS syndrome.

Cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) syndrome (OMIM# 601338) is a rare autosomal dominant disorder characterized by episodic, fever-induced ataxic encephalopathy in childhood with residual symptoms. All identified patients have the same heterozygous missense variant c.2452G>A (p.

The extent of intestinal failure-associated liver disease in patients referred for intestinal rehabilitation is associated with increased mortality: an analysis of the Pediatric Intestinal Failure Consortium database.

Background: The advent of regional multidisciplinary intestinal rehabilitation programs has been associated with improved survival in pediatric intestinal failure. Yet, the optimal timing of referral for intestinal rehabilitation remains unknown. We hypothesized that the degree of intestinal failure-associated liver disease (IFALD) at initiation of intestinal rehabilitation would be associated with overall outcome.

Partnerships for Global Child Health.

Child mortality remains a global health challenge and has resulted in demand for expanding the global child health (GCH) workforce over the last 3 decades. Institutional partnerships are the cornerstone of sustainable education, research, clinical service, and advocacy for GCH. When successful, partnerships can become self-sustaining and support development of much-needed training programs in resource-constrained settings.

Variceal Hemorrhage and Adverse Liver Outcomes in Patients With Cystic Fibrosis Cirrhosis.

Objectives: Cirrhosis occurs in 5% to 10% of cystic fibrosis (CF) patients, often accompanied by portal hypertension. We analyzed 3 adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver-related death (LD), and risk factors for these in CF Foundation Patient Registry subjects with reported cirrhosis.

Methods: We determined 10-year incidence rates for VB, LT, LD, and all-cause mortality (ACM), and examined risk factors using competing risk models and Cox-proportional hazard regression.

Nutritional Adequacy of the Specific Carbohydrate Diet in Pediatric Inflammatory Bowel Disease.

Introduction: The specific carbohydrate diet (SCD) is an exclusion diet used as a therapy in inflammatory bowel disease. The aim of this study was to evaluate the nutritional adequacy of the SCD.

Methods: Prospective dietary data for 12 weeks were analyzed for pediatric patients on the SCD.

Impact of processing methods on urinary biomarkers analysis in neonates.

Background: In neonates, the validation of urinary biomarkers to diagnose acute kidney injury is a rapidly evolving field. The neonatal population poses unique challenges when assessing the collection, storage, and processing of urinary samples for biomarker analysis. Given this, establishing optimal and consistent sample processing in this population for meaningful use in ongoing clinical trials is important.

Development and Initial Validation of the Macrophage Activation Syndrome/Primary Hemophagocytic Lymphohistiocytosis Score, a Diagnostic Tool that Differentiates Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome.

Objective: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis.

Study Design: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample.

Nutritional Therapy in Very Early-Onset Inflammatory Bowel Disease: A Case Report.

Inflammatory bowel disease (IBD) is a chronic inflammatory disease of the gastrointestinal tract caused by a dysregulated immune response to the fecal microbiota. Very early-onset inflammatory bowel disease (VEO-IBD) refers to a subgroup of pediatric patients with IBD diagnosed before 6 years of age. This subgroup is often characterized by increased severity, aggressive progression, strong family history of IBD, and often poor response to conventional treatments.

Enhancing Comparative Effectiveness Research With Automated Pediatric Pneumonia Detection in a Multi-Institutional Clinical Repository: A PHIS+ Pilot Study.

Background: Community-acquired pneumonia is a leading cause of pediatric morbidity. Administrative data are often used to conduct comparative effectiveness research (CER) with sufficient sample sizes to enhance detection of important outcomes. However, such studies are prone to misclassification errors because of the variable accuracy of discharge diagnosis codes.