Inhaled dry powder mannitol in children with cystic fibrosis: A randomised efficacy and safety trial.

Introduction: Inhaled mannitol has beneficial effects on lung function, mucociliary clearance, quality of life and sputum properties. This trial examined the efficacy of inhaled mannitol in children with cystic fibrosis (CF).

Methods: The efficacy of inhaled mannitol in children with CF aged 6-17years was assessed in a phase 2, randomised, placebo-controlled crossover study.

Hypercholesterolaemia screening in Type 1 diabetes: a difference of opinion.

Aim: To assess cholesterol screening of children with Type 1 diabetes by diabetes professionals using a survey of current practice, given that National Institute of Health and Care Excellence guidelines on childhood Type 1 diabetes do not recommend cholesterol screening, yet the National Paediatric Diabetes Audit has an annual cholesterol measure (> 12 years) as a key outcome indicator.

Methods: An online survey was sent to 280 members of the Association of Children's Diabetes Clinicians to assess cholesterol screening practice in children.

Results: A total of 87 diabetes professionals (31%) responded.

An alternative sensor-based method for glucose monitoring in children and young people with diabetes.

Objective: To determine accuracy, safety and acceptability of the FreeStyle Libre Flash Glucose Monitoring System in the paediatric population.

Design, Setting And Patients: Eighty-nine study participants, aged 4-17 years, with type 1 diabetes were enrolled across 9 diabetes centres in the UK. A factory calibrated sensor was inserted on the back of the upper arm and used for up to 14 days.

A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis.

The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) surgery for revalidation of neonatal surgeons. The aim of this study was therefore to calculate the number of infants in the UK/Ireland with surgical NEC and describe outcomes that could be used for national benchmarking and counselling of parents. A prospective nationwide cohort study of every infant requiring surgical intervention for NEC in the UK was conducted between 01/03/13 and 28/02/14.

Variability of outcome reporting in Hirschsprung's Disease and gastroschisis: a systematic review.

Heterogeneity in outcome reporting limits identification of gold-standard treatments for Hirschsprung's Disease(HD) and gastroschisis. This review aimed to identify which outcomes are currently investigated in HD and gastroschisis research so as to counter this heterogeneity through informing development of a core outcome set(COS). Two systematic reviews were conducted.

A 6.5 mb deletion at 3q24q25.2 narrows Wisconsin syndrome critical region to a 750 kb interval: A potential role for MBNLI.

We report on a patient with a 6.5 Mb interstitial de novo deletion in 3q24q25.2, characterized by array CGH.

Altered functional brain connectivity in children and young people with opsoclonus-myoclonus syndrome.

Aim: Opsoclonus-myoclonus syndrome (OMS) is a rare, poorly understood condition that can result in long-term cognitive, behavioural, and motor sequelae. Several studies have investigated structural brain changes associated with this condition, but little is known about changes in function. This study aimed to investigate changes in brain functional connectivity in patients with OMS.

Lifestyle treatments in cystic fibrosis: The NHS should not pay.

Lifestyle treatments can be defined as those which may have in impact on quality of life but do not affect health outcomes. Particular treatment options may be preferred by patients because they are for example, easier to use, take up less time or taste better. The impact on adherence needs to be considered.

Autosomal dominant SCN8A mutation with an unusually mild phenotype.

Background: Mutations in SCN8A, coding for the voltage-gated sodium channel Nav 1.6, have been described in relation to infantile onset epilepsy with developmental delay and cognitive impairment, in particular early onset epileptic encephalopathy (EIEE) type 13.

Case Report: Here we report an infant and his father with early onset focal epileptic seizures but without cognitive or neurological impairment in whom next generation sequence analysis identified a heterozygous mutation (c.

What Evidence Underlies Clinical Practice in Paediatric Surgery? A Systematic Review Assessing Choice of Study Design.

Objective: Identify every paediatric surgical article published in 1998 and every paediatric surgical article published in 2013, and determine which study designs were used and whether they were appropriate for robustly assessing interventions in surgical conditions.

Methods: A systematic review was conducted according to a pre-specified protocol (CRD42014007629), using EMBASE and Medline. Non-English language studies were excluded.

Accuracy of prenatal detection of tracheoesophageal fistula and oesophageal atresia.

Aims: This study aims to determine the rate of prenatal detection of tracheoesophageal fistula and oesophageal atresia (TOF/OA), by identifying a small or absent stomach bubble with or without polyhydramnios, on the prenatal ultrasound scans (USS).

Methods: A retrospective study of prenatal ultrasound findings of babies with a prenatal and postnatal diagnosis of TOF/OA born between 1st January 2004 and 31st December 2013 was undertaken.

Results: A total of 58 babies were born with TOF/OA.

Management of children with type 1 diabetes during illness: a national survey.

Aim: Adequate sick-day management at home can reduce the risk of progression to diabetic ketoacidosis and admission to hospital. The aim of this project was to review the management of diabetes during illness.

Method: The Association of Children's Diabetes Clinicians (ACDC) carried out a questionnaire survey of all paediatric diabetes units.

Diabetic ketoacidosis in an adolescent and young adult population in the UK in 2014: a national survey comparison of management in paediatric and adult settings.

Aims: To assess the management of diabetic ketoacidosis in young people, which differs in the UK between paediatric and adult services, and to evaluate outcomes and extent to which national guidelines are used.

Methods: A standardized questionnaire was sent to all paediatric and adult diabetes services in England, requesting details of all diabetic ketoacidosis admissions in young people aged > 14 years in paediatric services ('paediatric' patients), and in young adults up to the age of 22 years in adult services ('adult' patients).

Results: A total of 64 adult patients aged ≤ 22 years (mean age 19.

Slip-Knot-Loop Suture for Continuous Suturing in Laparoscopy.

Aims: One of the challenges of laparoscopic surgery is the time required to perform intracorporeal knots. This can result in considerably longer operating times when compared with the open approach. An example of this is pediatric laparoscopic pyeloplasty, where extensive laparoscopic suturing is required.

A simple scoring system to train surgeons in basic laparoscopic skills.

Aim: To develop and validate a scoring system for a training assessment tool using a box trainer which can objectively demonstrate progression in laparoscopic skills.

Method: 170 assessments were performed over a 5-year period by doctors working in a busy paediatric surgical department. Each participant was scored based on experience and then undertook six laparoscopic tasks in a box trainer in a dry skills lab.

Protocol for systematic review of evidence on the determinants and influence of early glycaemic control in childhood-onset type 1 diabetes.

Background: Landmark studies in adult-onset type 1 diabetes (T1D) populations indicate that improved glycaemic control through use of intensive insulin therapy is strongly associated with reduced risk for the development of diabetes-related complications and mortality in later years. However, it is unclear whether these associations can be extrapolated to childhood-onset T1D, given the influence of other important biological and psychosocial determinants of glycaemic control, particularly during adolescence. The aims of the review are (1) to investigate the impact of early glycaemic control (within the first 2 years after diagnosis) on subsequent glycaemic trends and risk of complications during the life course of childhood-onset T1D and (2) to identify the predictors of early glycaemic control in children and young people (0-25 years).

A Systematic Review of Posttraumatic Growth in Survivors of Interpersonal Violence in Adulthood.

This review critically evaluates the literature on posttraumatic growth in survivors of interpersonal violence, integrating the findings from 12 quantitative and 4 qualitative studies. The following databases were searched using predetermined terms: AMED, EMBASE, MEDLINE, PsycINFO, BNI, CINAHL, and Web of Knowledge. The review's findings suggest that the mean prevalence of growth in interpersonal violence survivors is around 71% (range 58-99%).

Global health: A lasting partnership in paediatric surgery.

Background: To emphasise the value of on-going commitment in Global Health Partnerships.

Materials And Methods: A hospital link, by invitation, was set up between United Kingdom and Tanzania since 2002. The project involved annual visits with activities ranging from exchange of skill to training health professionals.

Optimising nutrition to improve growth and reduce neurodisabilities in neonates at risk of neurological impairment, and children with suspected or confirmed cerebral palsy.

Background: Neurological impairment is a common sequelae of perinatal brain injury. Plasticity of the developing brain is due to a rich substrate of developing neurones, synaptic elements and extracellular matrix. Interventions supporting this inherent capacity for plasticity may improve the developmental outcome of infants following brain injury.

Type 2 diabetes presenting with hyperglycaemic hyperosmolar state in an adolescent renal transplant patient.

Hyperglycaemic hyperosmolar state (HHS) is a life-threatening condition rarely seen in paediatrics. It is becoming increasingly recognised with the growing incidence of childhood type 2 diabetes mellitus (T2DM). We present a 16-year-old boy with Bardet-Biedl syndrome, with comorbidities including chronic renal impairment requiring renal transplant, isolated growth hormone (GH) deficiency and obesity, who presented on routine follow-up with new onset T2DM and in HHS.