Modified Crohn's Disease Exclusion Diet Maintains Remission in Pediatric Crohn's Disease; Randomized Controlled Trial.

Background & Aims: The Crohn's Disease (CD) Exclusion Diet (CDED)+Partial Enteral Nutrition (PEN) is effective for inducing remission in mild-moderate CD. We assessed whether a 2-week course of Exclusive Enteral Nutrition (EEN), followed by CDED+PEN is superior to 8 weeks of EEN in sustaining clinical remission at week 14 in mild-to-severe CD and if CDED+PEN can maintain remission to week 24.

Methods: This international, multicenter, randomized-controlled trial compared 2-weeks of EEN (Modulen®IBD) followed by 3 phases of the CDED+PEN (henceforth CDED) to 8 weeks of EEN, followed by PEN with free diet up to week 24 (henceforth EEN).

The Cognitive Profile in Adolescents With Anorexia Nervosa and the Relationship With Autism and ADHD: A Pilot Study.

Objective: We aimed to examine the cognitive profile in adolescents with anorexia nervosa (AN) and its association with traits of autism spectrum disorder (ASD) and ADHD. In addition, resemblance in the cognitive profile between youths with AN and their parents was explored.

Methods: Adolescent females with acute AN (n = 20) and a healthy comparison group (n = 28) completed neuropsychological tasks of set-shifting (Trail making test, Wisconsin Card Sorting Test) and central coherence (Rey Complex Figures Task, Group Embedded Figures Test, object assembly subtest).

Impact of Race on the Outcomes of Retinoblastoma Treated With Primary Enucleation: A Global Study of 1426 Patients.

Background: To evaluate the clinical presentation, pathological features and outcomes of retinoblastoma based on the race of origin in a global cohort of patients.

Methods: Retrospective collaborative study of 1426 patients who underwent primary enucleation for retinoblastoma.

Results: Patients were grouped into Caucasians (n = 231, 16%), Asians (n = 841, 59%), Hispanics (n = 226, 16%), Arabs (n = 96, 7%) and Others (Africans, African Americans, Indigenous Australians; n = 32, 2%) cohorts.

Tranexamic acid in hip and spine surgery for children with cerebral palsy - a PRISMA-compliant scoping review.

Many children with cerebral palsy (CP) are frail and require major hip and/or spine surgeries associated with substantial blood loss. Tranexamic acid (TXA) is commonly used to reduce blood loss, but there is uncertainty around the optimal dose and timing of administration. There have been reviews in sub-populations and specific dosing regimens, but a broad overview of the available literature is lacking.

Vascular Ehlers-Danlos syndrome in children: evaluating the importance of diagnosis and follow-up during childhood.

Vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder predominantly caused by pathogenic COL3A1 variants. Characteristic arterial and intestinal fragility and generalised severe tissue friability can lead to clinical events from childhood. We highlight a paucity of literature regarding children diagnosed with vEDS, possibly explained by a restraint in predictive testing, and present data on 63 individuals (23 index cases) with a clinical and genetic diagnosis of vEDS in childhood (<18 years) to address this.

Improving outcomes for very preterm babies in England: does place of birth matter? Findings from OPTI-PREM, a national cohort study.

Objective: Babies born between 27 and 31 weeks of gestation contribute substantially towards infant mortality and morbidity. In England, their care is delivered in maternity services colocated with highly specialised neonatal intensive care units (NICU) or less specialised local neonatal units (LNU). We investigated whether birth setting offered survival and/or morbidity advantages to inform National Health Service delivery.

Trends in the survival of very preterm infants between 2011 and 2020 in France.

Objective: The objective is to evaluate changes in survival to discharge of liveborn infants less than 32 weeks' gestational age (GA) in France, where the latest available data on very preterm survival at a national-level are from the EPIPAGE-2 cohort in 2011.

Design: Population-based cohort study.

Setting: Metropolitan France in 2011, 2015 and 2020.

A stratified treatment algorithm in psychiatry: a program on stratified pharmacogenomics in severe mental illness (Psych-STRATA): concept, objectives and methodologies of a multidisciplinary project funded by Horizon Europe.

Schizophrenia (SCZ), bipolar (BD) and major depression disorder (MDD) are severe psychiatric disorders that are challenging to treat, often leading to treatment resistance (TR). It is crucial to develop effective methods to identify and treat patients at risk of TR at an early stage in a personalized manner, considering their biological basis, their clinical and psychosocial characteristics. Effective translation of theoretical knowledge into clinical practice is essential for achieving this goal.

Extreme Heat Stress and Unplanned Hospital Admissions.

Objectives: The burden and health impact of heat stress on child hospitalization is limited. This study aims to investigate associations between extreme heat stress exposure based on a Universal Thermal Climate Index (UTCI), emergency department (ED) visits, and ED visits that translate into unplanned hospital admissions.

Methods: This population-based case-crossover study included all ED visits and unplanned hospital admissions among children and adolescents aged 0 to 18 years from New South Wales, Australia, from July 2001 to June 2020.

CRKL Gene Deletion in Familial Zinner (OSVIRA) and OHVIRA Syndromes.

We present the first description of a family in which 2 siblings show alternative expression of CRKL gene deletion as the phenotypes of Zinner (OSVIRA, obstructed seminal vesicle and ipsilateral renal agenesis) and OHVIRA (obstructed hemivagina with an ipsilateral renal anomaly) syndromes. The male infant with Zinner syndrome and his sister aged 5 years with OHVIRA syndrome both have a paternally inherited 703-kb deletion at chromosome 22q11.21 that includes CRKL.

An Evaluation of Whether Routine QTc Interval Screening Is Necessary Prior to Starting ADHD Medications: Experience from a Large Retrospective Study.

Background/objectives: Routine screening electrocardiograms (ECGs) prior to starting medications for attention-deficit/hyperactivity disorder (ADHD) remain controversial. This real-world study assessed corrected QT (QTc) interval data from pediatric patients who had a baseline ECG performed prior to initiating treatment with ADHD medications and ≥6 months of clinical follow-up.

Methods: A retrospective chart review of children aged 2-18 years diagnosed with ADHD with/without autism spectrum disorder (ASD) at child neurology clinics in Jordan (June 2019 and June 2021) was performed, and children were prescribed with ADHD medications to manage symptoms.

Newborn Screening for Six Primary Conditions in a Clinical Setting in Morocco.

Unlabelled: Newborn screening (NBS) represents an important public health measure for the early detection of specified disorders; such screening can prevent disability and death, not only from metabolic disorders but also from endocrine, hematologic, immune, and cardiac disorders. Screening for critical congenital conditions affecting newborns' health is a great challenge, especially in developing countries such as Morocco, where NBS program infrastructure is lacking. In addition, the consanguinity rate is high in Morocco.

Prevalence of anxiety symptoms and associated factors at 2 months postpartum, results from a 2021 French national prospective cohort study.

Background: Postpartum anxiety (PPA) symptoms have harmful effects on child development and mother-infant interactions. Accordingly, in-depth knowledge of associated risk factors is crucial for prevention policies. This study aimed to estimate PPA symptom prevalence at 2 months and to identify associated risk factors in a representative sample of all women who gave birth in France in 2021, and in two subgroups: women with no postpartum depression (PPD) symptoms, and those with no history of mental health care.

Empowering professionals: a multidisciplinary approach to supporting people seeking asylum and refugees.

People seeking asylum and refugees living in initial accommodation in the UK can experience significant barriers to accessing care. They often have complex health and social needs and may experience fragmentation in the delivery of services. The Respond Integrated Refugee Health Service, hosted by University College London Hospital and operating across North Central London, has been created to address some of these challenges.

Commentary on the 2021 update of the KDIGO clinical practice guideline for management of blood pressure in chronic kidney disease.

The 2021 KDIGO clinical practice guideline for the management of blood pressure (BP) in chronic kidney disease (CKD) provided significant practice-changing recommendations for the care of both adult and paediatric CKD patients not receiving dialysis. The purpose of this review is to contextualise these recommendations and evaluate their applicability to the Australian and New Zealand context. Key updates presented in this guideline relate to measurement techniques, with a strong recommendation for standardised office BP measurement, as opposed to routine office BP measurement.

Incidence, mortality, and global burden of retinoblastoma in 204 countries worldwide from 1990 to 2021: Data and systematic analysis from the Global Burden of Disease Study 2021.

Background: Retinoblastoma (Rb), the primary intraocular malignancy in children, poses significant risks, yet its overall burden remains inadequately assessed. This study aims to analyze global Rb trends using Global Burden of Disease, Injuries, and Risk Factors study (GBD) 2021 data.

Methods: GBD 2021 data was analyzed to assess Rb incidence, mortality, and disability-adjusted life years (DALYs) from 1990 to 2021.

Hemostatic Outcome Definitions in Pediatric Extracorporeal Membrane Oxygenation: Challenges in Cohorts From Rotterdam (2019-2023) and Melbourne (2016-2022).

Objectives: To determine if a priori standardization of outcome hemostatic definitions alone was adequate to enable useful comparison between two cohorts of pediatric extracorporeal membrane oxygenation (ECMO) patients, managed according to local practice and protocol.

Design: Comparison of two separate prospective cohort studies performed at different centers with standardized outcome definitions agreed upon a priori.

Setting: General and cardiac PICUs at the Royal Children's Hospital (RCH) in Melbourne, Australia, and the Sophia Children's Hospital (SCH) in Rotterdam, The Netherlands.

Resuscitation in Paediatric Septic Shock Using Vitamin C and Hydrocortisone (RESPOND): The RESPOND Randomized Controlled Trial Protocol.

Objectives: Pediatric sepsis results in significant morbidity and mortality worldwide. There is an urgent need to investigate adjunctive therapies that can be administered early. We hypothesize that using vitamin C combined with hydrocortisone increases survival free of inotropes/vasopressors support until day 7 compared with standard care.

Allergen Specific IgE is a Stronger Predictor of Remission Following Peanut Oral Immunotherapy Than Age in Children Aged 1-10 Years.

Background: Remission is the desired outcome following OIT as it allows individuals to discontinue treatment and eat the allergen freely. Early initiation of OIT in infants and toddlers has been embraced as an approach to increase the likelihood of remission. However, there is no high-quality evidence supporting younger age as an independent factor driving remission; available studies are limited by small samples of younger subjects and lack of adjustment for confounding covariates, particularly peanut-specific IgE (sIgE) levels which is closely correlated with age.

Genotype-Guided Asthma Treatment Reduces Exacerbations in Children: Meta-Analysis of Two RCTs.

Background: Long-acting beta2-agonists (LABA) in combination with inhaled corticosteroids (ICS) are commonly used to treat asthma, however, some children lack response to the addition of LABA. This might be partially due to the presence of the Arg16Gly polymorphism, encoded by rs1042713 G>A in the ADRB2 gene. Carrying the A allele (Arg16) at this variant has been associated with an increased risk of exacerbations despite LABA treatment.

Sleep quality in children with hepatic glycogen storage diseases, a prospective observational pilot study.

Background: Hepatic glycogen storage diseases (GSDs) are characterised by enzyme defects affecting liver glycogen metabolism, where carbohydrate supplementation to prevent overnight hypoglycaemia is common. Concerns around sleep quality in hepatic GSDs relate to emerging evidence that overnight dysglycaemia impacts sleep quality.

Methods: This prospective observational study reported sleep quality and duration in children with hepatic GSDs over 7 days utilising: actigraphy (Actiwatch 2 by Phillips Respironics), sleep diaries, proxy reported age-appropriate sleep and quality-of-life (QoL) questionnaires, in the context of nocturnal glycaemic profiles continuous glucose monitor (CGM, Dexcom G6) and nocturnal dietary management strategies.

Health-related Quality of Life in children and adolescents treated for acute lymphoblastic leukaemia (ALL), compared with healthy peers: a longitudinal study of early survivorship.

Purpose: Survival for childhood Acute Lymphoblastic Leukaemia (ALL) has surpassed 90%, making quality of survival an important endpoint in treatment outcomes. This study examined health-related quality of life (HRQoL) trajectories in early survivorship for patients post-ALL treatment compared with a matched group of healthy peers, and explored the association of individual factors (age, sex) and treatment intensity with HRQoL outcomes.

Methods: Eighty-three paediatric patients aged 4-16 years who recently completed treatment for ALL were recruited to the study, alongside 53 age- and sex-matched healthy children.

The Role of Neutrophils in Allergic Disease.

Neutrophils are short-lived cells of the innate immune system and represent 50-70% of the circulating leucocytes. Their primary role is antimicrobial defence which they accomplish through rapid migration to sites of inflammation followed by phagocytosis, degranulation and the release of neutrophil extracellular traps (NETosis). While previously considered terminally-differentiated cells, they have been shown to have great adaptability and to play a role in conditions ranging from cancer to autoimmunity.

Socio-economic disparities in the psychosocial and economic impacts of the COVID-19 pandemic on children and young people in Australia.

Aims: While COVID-19 no longer presents a global health emergency, the indirect (non-infection) impacts of the pandemic may exacerbate health inequalities in years to come. We examined the socio-economic distribution of the impacts of the pandemic on the psychosocial and economic well-being of children, young people and their families.

Methods: The CoRonavIruS Health Impact Survey (CRISIS) was conducted in seven existing longitudinal cohorts, together involving n = 3072 participants aged 0-19 years.

How do patients determine when their inhaler is empty? Insights from an analysis of returned inhalers and a patient survey.

Background: Inhalers are widely used for the management of asthma and chronic obstructive pulmonary disease. However, there is little knowledge about the extent to which an inhaler is used and when it is disposed of, despite the implications for an individual's health (when used beyond the recommended number of doses (overused)), and medicine wastage, healthcare costs and the environment (when discarded with remaining doses (underused)). To explore inhaler use, we assessed the number of doses remaining in pressurised metered-dose inhalers (pMDIs) returned via a Chiesi Inhaler Recycling scheme.