Systematic Evaluation of IMU Sensors for Application in Smart Glove System for Remote Monitoring of Hand Differences.

Human hands have over 20 degrees of freedom, enabled by a complex system of bones, muscles, and joints. Hand differences can significantly impair dexterity and independence in daily activities. Accurate assessment of hand function, particularly digit movement, is vital for effective intervention and rehabilitation.

Quantifying Additional Procedures in Functionally Single-Ventricle Disease: A National Cohort Study.

Background: Given their importance as a metric for health care evaluation, this study's aim was to evaluate the rates of surgical and catheter reinterventions for children with functionally single-ventricle (f-SV) congenital heart disease (CHD) undergoing staged palliation.

Methods: We undertook a retrospective cohort study of children born with f-SV CHD between 2000 and 2018 in England and Wales, using the national registry, with survival ascertained in 2020. Competing risk analysis was used to describe the incidence of additional procedures that occurred first, during follow-up, accounting for competing events of death or transplantation.

Patient Source of Referral Is a Key Determinant of Subsequent Retention in Care for Young Chronic Hepatitis B Patients.

Hepatitis B elimination objectives can only be realised if new patient linkage to care is matched by long-term patient retention in care. We previously showed in adult chronic hepatitis B (CHB) patients that retention in care was inferior in younger patients and in patients from non-Asian ethnicities. The present study explores further the rates and determinants of loss to follow-up in a cohort of 271 young patients (aged 16-21 years at baseline).

Building evidence-based interventions to improve staff well-being in paediatric critical care using the behaviour change wheel.

Background: Research has demonstrated that staff working in Paediatric Critical Care (PCC) experience high levels of burnout, post-traumatic stress and moral distress. There is very little evidence of how this problem could be addressed.

Aim: To develop evidence-based, psychologically informed interventions designed to improve PCC staff well-being that can be feasibility tested on a large scale.

Traumatic head injuries in children: demographics, injury patterns, and outcomes in Saudi Arabia.

Background: Traumatic head injuries (THIs) are among the leading cause of mortality and intensive care unit (ICU) admission in children worldwide. Most of the published literature concerning THIs arises predominantly from North America and Europe. However, only limited data about the incidence, characteristics and impact on children in Saudi Arabia exists.

Is there any role for an atrial septal defect in off-loading a borderline left ventricle?

A small atrial septal defect with right-to-left shunt is useful for off-loading a dysfunctional right ventricle postoperatively. However, an atrial septal defect with left-to-right shunt may not be as useful for a dysfunctional left ventricle. Experimental data are limited at present.

Oral health care pathways for patients with epidermolysis bullosa: A position statement from the European reference network for rare skin diseases.

Background: Inherited epidermolysis bullosa (EB) comprises a group of genetic disorders characterized by skin fragility and unique oral features. It requires interdisciplinary care from several health professionals, including oral health teams. Modern dentistry encompasses a wide range of therapeutic options performed by specialists from different fields.

Age distribution of retinoblastoma tumours in familial disease.

Background: 40% of children with retinoblastoma have an RB1 gene mutation identified, known as heritable retinoblastoma. It is important to undertake active surveillance (screening) of relatives of those with identified RB1 gene mutations and ensure ongoing surveillance to monitor for new tumour formation or recurrences. Current guidance is to screen patients up to the age of 7 years old.

Intraocular Surgery for Retinoblastoma: An Evaluation of Current Evidence.

Background: Retinoblastoma (Rb) is the most common intraocular malignancy of childhood. The prognosis in Rb directly relates to the spread of disease beyond the eye, particularly to the central nervous system. Therefore, until the recent past, surgically entering an eye with active or recently active Rb was absolutely contraindicated in most centres around the world due to the risk of iatrogenic extraocular spread.

The Use of Automated Insulin Delivery around Physical Activity and Exercise in Type 1 Diabetes: A Position Statement of the European Association for the Study of Diabetes (EASD) and the International Society for Pediatric and Adolescent Diabetes (ISPAD).

Regular physical activity and exercise (PA) are cornerstones of diabetes care for individuals with type 1 diabetes. In recent years, the availability of automated insulin delivery (AID) systems has improved the ability of people with type 1 diabetes to achieve the recommended glucose target ranges. PA provides additional health benefits but can cause glucose fluctuations, which challenges current AID systems.

A new clinical index scale for measuring secondary alveolar bone grafting success based on canine eruption.

Objectives: To develop a reproducible, reliable clinical index of alveolar bone grafting (ABG) outcome based on the eruption position of the cleft canine tooth and determine the association between eruption position and radiographic outcome at 6 months post-grafting.

Methods: Children with complete, non-syndromic, unilateral/bilateral cleft lip and palate in the West of Scotland were identified. Post-ABG radiographic outcome (Kindelan index) and canine eruption position in children with a cleft of the alveolus who had undergone ABG were documented.

The use of automated insulin delivery around physical activity and exercise in type 1 diabetes: a position statement of the European Association for the Study of Diabetes (EASD) and the International Society for Pediatric and Adolescent Diabetes (ISPAD).

Regular physical activity and exercise (PA) are cornerstones of diabetes care for individuals with type 1 diabetes. In recent years, the availability of automated insulin delivery (AID) systems has improved the ability of people with type 1 diabetes to achieve the recommended glucose target ranges. PA provide additional health benefits but can cause glucose fluctuations, which challenges current AID systems.

Stereoelectroencephalography (SEEG)-guided insula resections: is it "Reily" worth it?

Objective: Stereoelectroencephalography (SEEG) is widely used to characterise epileptic networks and guide resection in paediatric epilepsy surgery programmes. The insula, with its extensive connectivity with temporal and extratemporal structures, has increasingly been seen as a possible surgical target. We report our seizure outcomes after SEEG-guided resection of the insula in a paediatric cohort.

Microbiological analysis of donor human milk over seven years from the Hearts Milk Bank (United Kingdom).

When maternal milk is unavailable, donor human milk (DHM) from human milk banks (HMBs) is the optimal alternative, as recommended by the World Health Organisation. The microbiota of DHM could contain opportunistic pathogens, which means rigorous microbiological screening for DHM, prior to pasteurisation, is recommended to safeguard recipients. Here, an analysis of 6863 DHM samples from 1419 donors at the Hearts Milk Bank between 2017 and 2023 showed approximately 70.

Reverse transcriptase inhibitors in Aicardi-Goutières syndrome: A crossover clinical trial.

Aim: To extend the findings of a previous clinical trial suggesting combined abacavir (ABC), lamivudine (3TC), and zidovudine (AZT) reduces type I interferon (IFN) signalling in Aicardi-Goutières syndrome (AGS).

Method: This was an open label, non-placebo-controlled phase II clinical trial (NCT04731103) in patients less than 16 years with any of five AGS genotypes. The effect of ABC or 3TC individually, or of combined ABC + 3TC + AZT, on IFN-stimulated gene (ISG) expression (primary outcome) and IFN-alpha protein (secondary outcome) in blood was assessed.

Evaluating Renal Disease in Pediatric-Onset Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Disease Course, Outcomes, and Predictors of Outcome.

Objective: We aimed to study the disease course, outcomes, and predictors of outcome in pediatric-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) affecting the kidneys.

Methods: Patients eligible for this study had a diagnosis of granulomatosis with polyangiitis (GPA), microscopic polyangiitis, or ANCA-positive pauci-immune glomerulonephritis, were 18 years or younger at diagnosis, had renal disease defined by biopsy or dialysis dependence, and had clinical data at diagnosis and at either 12 or 24 months. Ambispective data from A Registry for Children with Vasculitis/Pediatric Vasculitis Initiative Registry was used.

Cranial vault expansion in treatment of paediatric idiopathic intracranial hypertension.

Introduction: Idiopathic intracranial hypertension (IIH) is a rare clinical entity in the paediatric population. Clinical presentation is mostly similar to adult counterpart and can include headaches, vomiting, papilloedema, deterioration in visual acuity or fields, and 6th cranial nerve palsy, leading to significant morbidity. Therapeutic lumbar puncture and medical treatment with acetazolamide are usually the first-line treatments.

Pseudoachondroplasia associated with os odontoideum and retro-odontoid mass: case-based update.

Purpose: Pseudoachondroplasia is a rare skeletal dysplasia caused by a mutation in the COMP gene. Infants with pseudoachondroplasia present with rhizomelic dwarfism. Pseudoachondroplasia can resemble achondroplasia, which also presents with a phenotype of rhizomelic dwarfism.

Cost-effectiveness of high flow nasal cannula therapy versus continuous positive airway pressure for non-invasive respiratory support in paediatric critical care.

Background: High flow nasal cannula therapy (HFNC) and continuous positive airway pressure (CPAP) are two widely used modes of non-invasive respiratory support in paediatric critical care units. The FIRST-ABC randomised controlled trials (RCTs) evaluated the clinical and cost-effectiveness of HFNC compared with CPAP in two distinct critical care populations: acutely ill children ('step-up' RCT) and extubated children ('step-down' RCT). Clinical effectiveness findings (time to liberation from all forms of respiratory support) showed that HFNC was non-inferior to CPAP in the step-up RCT, but failed to meet non-inferiority criteria in the step-down RCT.

Management of Ganglioneuroma and Ganglioneuroblastoma Intermixed: A United Kingdom Children's Cancer and Leukaemia Group (UK CCLG) Nationwide Study Report.

Background: Ganglioneuroblastoma intermixed (GNBi) and ganglioneuroma (GN) represent benign variants of peripheral neuroblastic tumours. While historically surgical resection was recommended, watchful active observation has become the accepted management for a subset of patients.

Objectives: To review clinical features, biology, natural history and management of a retrospective UK CCLG study cohort of GN and GNBi, and compare outcomes of patients treated with surgical resection or watchful active observation.

Clean Intermittent Catheterisation via a Mitrofanoff Is Superior to Long Term Suprapubic Catheters for Bladder Drainage.

Aim: Some children require long-term drainage of the bladder but do not tolerate clean intermittent catheterisation (CIC) urethrally. We aimed to compare long term suprapubic catheter (SPC) drainage vs Mitrofanoff conduit (allowing CIC) by comparing the survival of the drainage methods and rates of urinary tract infection (UTI).

Method: Retrospective review of a single surgeon's experience (2007-2023).

Long-term Survival of Bladder Augmentation is Influenced by its Shape and Mucosal Lining.

Introduction: Bladder augmentation in the UK has been largely by enterocystoplasty or ureterocystoplasty (UC). Ileocystoplasty can be simple patch placement (SPP), or formation of an ileal cup (IC). Urothelium is the "right" mucosa, whereas intestinal mucosa exhibits absorption, mucus production, malignancy.

UKHCDO gene therapy taskforce: Guidance for implementation of haemophilia gene therapy into routine clinical practice for adults.

Introduction: 2022 was a landmark year with two adeno-associated viral vectors (AAVs) receiving conditional marketing authorization from EMA for the treatment of persons with severe haemophilia A and severe to moderately severe haemophilia B and a third in 2024. Gene therapy is a transformative, irreversible treatment with long-lasting effects, necessitating development of new clinical pathways to ensure optimal outcomes.

Aim: To develop a consensus framework and service specification for delivery of AAV gene therapy for haemophilia in adults within the UK using the hub-and-spoke model proposed by the European Association of Haemophilia and Allied Disorders and the European Haemophilia Consortium.