Management of Gastroschisis: Results From the NETS2G Study, a Joint British, Irish, and Canadian Prospective Cohort Study of 1268 Infants.

Objective: In infants with gastroschisis, outcomes were compared between those where operative reduction and fascial closure were attempted ≤24 hours of age (PC), and those who underwent planned closure of their defect >24 hours of age following reduction with a pre-formed silo (SR).

Summary Of Background Data: Inadequate evidence exists to determine how best to treat infants with gastroschisis.

Methods: A secondary analysis was conducted of data collected 2006-2008 using the British Association of Pediatric Surgeons Congenital Anomalies Surveillance System, and 2005-2016 using the Canadian Pediatric Surgery Network.

A framework synthesis reviewing the impact of neonatal care unit admission on early caregiver-infant relationships.

Aims: To critically review and synthesize qualitative research pertaining to the establishment of early caregiver-infant relationships in the neonatal care unit (NCU).

Background: It is well-established that bonding and attachment, established across the prenatal and early childhood periods, affect child cognitive and behavioural development. Proximity, reciprocity, and commitment are key to the formation of these early relationships.

The correlation between breath acetone and blood betahydroxybutyrate in individuals with type 1 diabetes.

Ketone testing is an important element of the self-management of illness in type 1 diabetes. The aim of the present study was to see if a breath test for acetone could be used to predict quantitatively the levels of the ketone betahydroxybutyrate in the blood of those with type 1 diabetes, and thus be used as an alternative to capillary testing for ketones. Simultaneous capillary ketones and breath acetone were measured in 72 individuals with type 1 diabetes attending a diabetes clinic and on 9 individuals admitted to hospital with diabetic ketoacidosis.

Adjuvant tyrosine kinase inhibitor therapy improves outcome for children and adolescents with acute lymphoblastic leukaemia who have an ABL-class fusion.

Patients with an ABL-class fusion have a high risk of relapse on standard chemotherapy but are sensitive to tyrosine kinase inhibitors (TKI). In UKALL2011, we screened patients with post-induction MRD ≥1% and positive patients (12%) received adjuvant TKI. As the intervention started during UKALL2011, not all eligible patients were screened prospectively.

Five Applications of Narrative Exposure Therapy for Children and Adolescents Presenting With Post-Traumatic Stress Disorders.

Narrative exposure therapy (NET) is an individual therapeutic approach that has an emerging evidence base for children. It was initially trialed with refugee and asylum seeking populations, in low, middle and high-income settings, utilizing either lay or professional therapists. The results of treatment trials for PTSD in refugee children with NET (or the child "KIDNET" adaptation) demonstrates how this is an effective intervention, is scalable and culturally dexterous.

The identification of key indicators to drive quality improvement in obstetric anaesthesia: results of the Obstetric Anaesthetists' Association/National Perinatal Epidemiology Unit collaborative Delphi project.

A three-stage Delphi survey process was undertaken to identify the quality indicators considered the most relevant to obstetric anaesthesia. The initial quality indicators assessed were derived from national peer-reviewed publications and were divided into service provision, service quality and clinical outcomes. A range of stakeholders were invited to participate and divided into three panels: obstetric anaesthetists; other maternity care health professionals; and women who had used maternity services.

Multicenter Study of 342 Anorectal Malformation Patients: Age, Gender, Krickenbeck Subtypes, and Associated Anomalies.

Introduction:  Published studies based on Krickenbeck classification of anorectal malformations (ARMs) are still insufficient to assess the global as well as regional relative incidence of different ARM subtypes, gender distribution, and associated anomalies. The primary purpose of this study was to provide an estimate of those in Global Initiative for Children's Surgery (GICS) research group.

Materials And Methods:  We collected ARM data prospectively for 1 year from four institutes of different geographic locations.

Measurement of Salivary Adrenal-Specific Androgens as Biomarkers of Therapy Control in 21-Hydroxylase Deficiency.

Background: Monitoring of hormonal control represents a key part of the management of congenital adrenal hyperplasia (CAH). Monitoring strategies remain suboptimal because they rely on frequent blood tests and are not specific for adrenal-derived hormones. Recent evidence suggests the crucial role of adrenal-specific 11-oxygenated-C19 androgens in the pathogenesis of CAH.

Utility and safety of plasma exchange in paediatric neuroimmune disorders.

Aim: Our aim was to ascertain the indications, side effects, and outcomes in children receiving therapeutic plasma exchange (TPE) for neurological disorders.

Method: Medical records were retrospectively reviewed for 58 consecutive children (age ≤16y) undergoing 67 courses of TPE across four tertiary centres. Patient characteristics, treatment schedules, complications, and outcomes were analysed.

Does age matter? A comparison of health-related quality of life issues of adolescents and young adults with cancer.

Objective: Health-related quality of life (HRQoL) concerns of adolescents and young adults (AYAs) aged 14-25 years were compared with those of older adults (26-60 years) with cancer.

Methods: AYAs and older adults receiving curative intent treatment or supportive palliative care for cancer were recruited from eight research centres across Europe. Participants used a rating scale to score the relevance and importance of a list of 77 issues covering 10 areas of HRQoL concern: symptoms; activity restrictions; social; emotional; body image; self-appraisals; outlook on life; lifestyle; treatment-related and life beyond treatment.

Introduction to the Craniofacial Collaboration UK: A Developmental Screening Protocol at the United Kingdom's Four Highly Specialized Craniofacial Centers.

The clinical psychologists at the 4 highly specialized craniofacial centers in the United Kingdom have developed a systematic, developmental screening protocol, called the Craniofacial Collaboration UK (CC-UK). Previous systematic reviews of the literature into the developmental sequelae of single-suture craniosynostosis have identified a number of methodological flaws which the CC-UK seeks to avoid. This study presents an introduction to the rationale for its development, as well as the methodology of the CC-UK.

Surveillance for variant CJD: should more children with neurodegenerative diseases have autopsies?

Objectives: To report investigations performed in children with progressive neurodegenerative diseases reported to this UK study.

Design: Since 1997 paediatric surveillance for variant Creutzfeldt-Jakob disease (vCJD) has been performed by identifying children aged less than 16 years with progressive intellectual and neurological deterioration (PIND) and searching for vCJD among them.

Setting: The PIND Study obtains case details from paediatricians who notify via the British Paediatric Surveillance Unit.

Early and Late Histological and Ultrastructural Findings in Resected Infantile Capillary Hemangiomas Following Treatment with Topical Beta-Blocker Timolol Maleate 0.5.

Background: Infantile capillary hemangiomas (IHs) affect approximately 4-5% of infants. The systemic nonselective β-adrenergic antagonist, propranolol, has become the standard first-line treatment for severe IHs. The topical β-antagonist, timolol maleate, has also demonstrated efficacy and safety in treating superficial and some deep capillary hemangiomas.

1q24 deletion syndrome. Two cases and new insights into genotype-phenotype correlations.

1q24q25 deletions cause a distinctive phenotype including proportionate short stature, microcephaly, brachydactyly, dysmorphic facial features and intellectual disability. We present a mother and son who have a 672 kb microdeletion at 1q24q25. They have the typical skeletal features previously described but do not have any associated intellectual disability.

Systematic review and meta-analysis comparing outcomes following orchidopexy for cryptorchidism before or after 1 year of age.

Background: Current guidelines recommend orchidopexy for cryptorchidism by 12 months of age, yet this is not universally adhered to. The aim of this systematic review and meta-analysis was to compare outcomes between orchidopexies performed before and after 1 year of age.

Methods: MEDLINE and Embase were searched (September 2015) using terms relating to cryptorchidism, orchidopexy and the outcomes of interest.

Development of a gastroschisis core outcome set.

Objective: Outcome reporting heterogeneity impedes identification of gold standard treatments for children born with gastroschisis. Use of core outcome sets (COSs) in research reduces outcome reporting heterogeneity and ensures that studies are relevant to patients. The aim of this study was to develop a gastroschisis COS.

Diagnosis of sporadic neurofibromatosis type 2 in the paediatric population.

Objective: Onset of symptoms in severe sporadic neurofibromatosis type 2 (NF2) is typically within childhood; however, there is poor awareness of presenting features in young children, potentially resulting in delayed diagnosis and poorer outcome. We have reviewed presentation of sporadic paediatric NF2 to raise awareness of early features, highlighting those requiring further investigation.

Design: Patients diagnosed with NF2 at age ≤16 and seen between 2012 and 2015 were notified via the British Paediatric Neurology Surveillance Unit or identified through the English NF2 service.

Navigating chronic pain: how taking a few wrong turns can miss sinister pathology.

Bone sarcomas are rare in childhood, and their presentation can often mimic more benign complaints or chronic musculoskeletal pain. Ewing sarcomas in particular are often diagnosed after a significant delay from the onset of symptoms. At a population level, a long diagnostic delay is not necessarily associated with worse survival , as tumours that display slow growth also tend to be less aggressive.

A survey of staffing levels in paediatric diabetes services throughout the UK.

Aims: To assess staffing levels of healthcare professionals involved in the care of children and young people with diabetes in the UK.

Methods: A web-based questionnaire was distributed to lead consultant paediatricians from all paediatric diabetes services in the UK between October and December 2014. Data on staffing levels and other aspects of diabetes services were collected and differences between the four nations of the UK and across the 10 English diabetes networks were explored.

One-year outcomes following surgery for necrotising enterocolitis: a UK-wide cohort study.

Objective: The objective was to describe outcomes and investigate factors affecting prognosis at 1 year post intervention for infants with surgical necrotising enterocolitis (NEC).

Design: Using the British Association of Paediatric Surgeons Congenital Anomalies Surveillance System, we conducted a prospective, multicentre cohort study of every infant reported to require surgical intervention for NEC in the UK and Ireland between 1 March 2013 and 28 February 2014. Association of independent variables with 1-year mortality was investigated using multivariable logistic regression analysis.