Comparative performance of head ultrasound and MRI in detecting preterm brain injury and predicting outcomes: A systematic review.

Aim: To systematically review the literature to compare the performance of head ultrasound (HUS) and magnetic resonance imaging (MRI) in their ability to detect brain injury and their predictive value for neurodevelopmental outcomes.

Methods: This was a systematic review based on literature search in three electronic databases (MEDLINE, EMBASE, Cochrane Library) and additional sources for studies on routine brain injury screening in preterm neonates published during 2000-May 2020. Studies were included if they reported on the comparative performance of HUS and MRI in detecting preterm brain injury and/or their predictive value for neurodevelopmental outcomes.

Atypical measures of diffusion at the gray-white matter boundary in autism spectrum disorder in adulthood.

Autism spectrum disorder (ASD) is a highly complex neurodevelopmental condition that is accompanied by neuroanatomical differences on the macroscopic and microscopic level. Findings from histological, genetic, and more recently in vivo neuroimaging studies converge in suggesting that neuroanatomical abnormalities, specifically around the gray-white matter (GWM) boundary, represent a crucial feature of ASD. However, no research has yet characterized the GWM boundary in ASD based on measures of diffusion.

Soluble Low-density Lipoprotein Receptor-related Protein 1 in Juvenile Idiopathic Arthritis.

Objectives: This study aimed to expand knowledge about soluble low-density lipoprotein receptor-related protein 1 (sLRP1) in juvenile idiopathic arthritis (JIA) by determining associations of sLRP1 levels in nonsystemic JIA patients with clinical and inflammatory biomarker indicators of disease activity.

Methods: Plasma sLRP1 and 44 inflammation-related biomarkers were measured at enrollment and 6 months later in a cohort of 96 newly diagnosed Canadian patients with nonsystemic JIA. Relationships between sLRP1 levels and indicators of disease activity and biomarker levels were analyzed at both visits.

Diplopia Following the Insertion of a Canalicular Bypass Tube: Etiology, Risk Factors, Management, and Outcomes.

Purpose: To report the etiology, management, and possible risk factors for diplopia after canalicular bypass surgery.

Methods: A multicenter retrospective, noncomparative case series of patients who developed diplopia following canalicular bypass surgery were assessed.

Results: Twenty-four cases of diplopia were identified across 12 institutions.

Umbilical vein infusion of prostaglandin I increases ductus venosus shunting of oxygen-rich blood but does not increase cerebral oxygen delivery in the fetal sheep.

Key Points: The ductus venosus (DV) is a dynamic fetal shunt that allows substrate-rich blood from the umbilical vein to bypass the hepatic circulation. In vitro studies suggest a direct role of prostaglandin I (PGI ) in the regulation of DV tone; however, the extent of this regulation has not been determined in utero. 4D flow and T oximetry magnetic resonance imaging can be combined to determine blood flow and oxygen delivery within the fetal circulation.

Elevated rates of autism, other neurodevelopmental and psychiatric diagnoses, and autistic traits in transgender and gender-diverse individuals.

It is unclear whether transgender and gender-diverse individuals have elevated rates of autism diagnosis or traits related to autism compared to cisgender individuals in large non-clinic-based cohorts. To investigate this, we use five independently recruited cross-sectional datasets consisting of 641,860 individuals who completed information on gender, neurodevelopmental and psychiatric diagnoses including autism, and measures of traits related to autism (self-report measures of autistic traits, empathy, systemizing, and sensory sensitivity). Compared to cisgender individuals, transgender and gender-diverse individuals have, on average, higher rates of autism, other neurodevelopmental and psychiatric diagnoses.

American College of Rheumatology Guidance for the Management of Pediatric Rheumatic Disease During the COVID-19 Pandemic: Version 1.

Objective: To provide clinical guidance to rheumatology providers who treat children with pediatric rheumatic disease (PRD) in the context of the coronavirus disease 2019 (COVID-19) pandemic.

Methods: The task force, consisting of 7 pediatric rheumatologists, 2 pediatric infectious disease physicians, 1 adult rheumatologist, and 1 pediatric nurse practitioner, was convened on May 21, 2020. Clinical questions and subsequent guidance statements were drafted based on a review of the queries posed by the patients as well as the families and healthcare providers of children with PRD.

Long-term effects of medical management on growth and weight in individuals with urea cycle disorders.

Low protein diet and sodium or glycerol phenylbutyrate, two pillars of recommended long-term therapy of individuals with urea cycle disorders (UCDs), involve the risk of iatrogenic growth failure. Limited evidence-based studies hamper our knowledge on the long-term effects of the proposed medical management in individuals with UCDs. We studied the impact of medical management on growth and weight development in 307 individuals longitudinally followed by the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD).

An OTX2-PAX3 signaling axis regulates Group 3 medulloblastoma cell fate.

OTX2 is a potent oncogene that promotes tumor growth in Group 3 medulloblastoma. However, the mechanisms by which OTX2 represses neural differentiation are not well characterized. Here, we perform extensive multiomic analyses to identify an OTX2 regulatory network that controls Group 3 medulloblastoma cell fate.

Co-occurring trajectories of anxiety and insistence on sameness behaviour in autism spectrum disorder.

Background: Children with autism spectrum disorder (ASD) have increased susceptibility to anxiety disorders. Variation in a common ASD symptom, insistence on sameness behaviour, may predict future anxiety symptoms.

Aims: To describe the joint heterogeneous longitudinal trajectories of insistence on sameness and anxiety in children with ASD and to characterise subgroups at higher risk for anxiety.

Epidermal growth factor receptor deficiency: Expanding the phenotype beyond infancy.

Transmembrane tyrosine kinase receptors represent a fundamental mechanism for transducing extracellular signals into the activation of signaling cascades responsible for intercellular communication, embryogenesis and tissue integrity. The epidermal growth factor receptor (EGFR) is a canonical member of this family, regarded for its dysregulated function in various malignancies. Here, we describe a young female born prematurely with friable and immature skin who developed chronic diarrhea, recurrent gastrointestinal and respiratory infections, as well as an ichthyotic and inflammatory papulopustular rash accompanied by alopecia.

Recognition of Girls on the Autism Spectrum by Primary School Educators: An Experimental Study.

Autism has long been considered a predominantly male condition. It is increasingly understood, however, that autistic females are under-recognized. This may reflect gender stereotyping, whereby symptoms are missed in females, because it is assumed that autism is mainly a male condition.

Mechanical Ventilation Duration, Brainstem Development, and Neurodevelopment in Children Born Preterm: A Prospective Cohort Study.

Objectives: To determine, in children born preterm, the association of mechanical ventilation duration with brainstem development, white matter maturation, and neurodevelopmental outcomes at preschool age.

Study Design: This prospective cohort study included 144 neonates born at <30 weeks of gestation (75 male, mean gestational age 27.1 weeks, SD 1.

Identification of Preschool Children with Mental Health Problems in Primary Care: Systematic Review and Meta-analysis.

Objective: Primary care practitioners determine access to care for many preschool children with mental health (MH) problems. This study examined rates of mental health (MH) problem identification in preschoolers within primary healthcare settings, related service use, and MH status at follow-up. The findings may inform evidence-based policy and practice development for preschool MH.

Advances in the molecular classification of pediatric brain tumors: a guide to the galaxy.

Central nervous system (CNS) tumors are the most common solid tumor in pediatrics, accounting for approximately 25% of all childhood cancers, and the second most common pediatric malignancy after leukemia. CNS tumors can be associated with significant morbidity, even those classified as low grade. Mortality from CNS tumors is disproportionately high compared to other childhood malignancies, although surgery, radiation, and chemotherapy have improved outcomes in these patients over the last few decades.

Associations of clinical and inflammatory biomarker clusters with juvenile idiopathic arthritis categories.

Objective: To identify discrete clusters comprising clinical features and inflammatory biomarkers in children with JIA and to determine cluster alignment with JIA categories.

Methods: A Canadian prospective inception cohort comprising 150 children with JIA was evaluated at baseline (visit 1) and after six months (visit 2). Data included clinical manifestations and inflammation-related biomarkers.

Analysis of Extreme Length of Stay Hospitalizations for Children and Youth in a Quaternary Care Hospital.

Length of stay (LOS) is an important issue for many healthcare organizations. In-patients with extreme LOS account for a disproportionately large percentage of hospital costs. Our analysis of over 15,000 pediatric hospital discharges at The Hospital for Sick Children (Toronto, Canada) between 2015 and 2016 revealed that the vast majority of patients with extreme LOS were discharged directly home, with only a minority receiving home-based services.

Effects of oxytocin administration on salivary sex hormone levels in autistic and neurotypical women.

Background: Oxytocin administration, which may be of therapeutic value for individuals with social difficulties, is likely to affect endogenous levels of other socially relevant hormones. However, to date, the effects of oxytocin administration on endogenous hormones have only been examined in neurotypical individuals. The need to consider multi-hormone interactions is particularly warranted in oxytocin trials for autism due to evidence of irregularities in both oxytocin and sex steroid systems.

Lower versus Traditional Treatment Threshold for Neonatal Hypoglycemia.

Background: Worldwide, many newborns who are preterm, small or large for gestational age, or born to mothers with diabetes are screened for hypoglycemia, with a goal of preventing brain injury. However, there is no consensus on a treatment threshold that is safe but also avoids overtreatment.

Methods: In a multicenter, randomized, noninferiority trial involving 689 otherwise healthy newborns born at 35 weeks of gestation or later and identified as being at risk for hypoglycemia, we compared two threshold values for treatment of asymptomatic moderate hypoglycemia.

Hospital resources do not predict accuracy of secondary trauma triage: A population-based analysis.

Background: The identification of patients who require transfer from non-trauma centers to trauma centers (secondary triage) is complicated by high rates of undertriage and overtriage. The objective of this study was to evaluate variations in secondary triage accuracy across non-trauma centers and identify factors associated with highly accurate secondary triage.

Methods: We performed a population-based study of injured patients who presented to non-trauma centers in a large regional trauma system.

The Challenges and Opportunities of Classifying Childhood Arthritis.

Purpose Of Review: Childhood arthritis is in need of a new system of classification, owing to deficiencies in the International League of Associations for Rheumatology (ILAR) criteria. We briefly review the history of classification of childhood arthritis, discuss the major criticisms of the current system, and highlight current initiatives to address those concerns.

Recent Findings: Recent studies in both pediatric and adult rheumatology into the biologic basis of disease as well as the clinical patterns of presentation have informed the efforts toward developing a new classification system.

Clinical and associated inflammatory biomarker features predictive of short-term outcomes in non-systemic juvenile idiopathic arthritis.

Objective: To identify early predictors of disease activity at 18 months in JIA using clinical and biomarker profiling.

Methods: Clinical and biomarker data were collected at JIA diagnosis in a prospective longitudinal inception cohort of 82 children with non-systemic JIA, and their ability to predict an active joint count of 0, a physician global assessment of disease activity of ≤1 cm, and inactive disease by Wallace 2004 criteria 18 months later was assessed. Correlation-based feature selection and ReliefF were used to shortlist predictors and random forest models were trained to predict outcomes.

Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases.

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.