Perinatal optimisation and predictive testing of in utero transfers and their outcome: A regional study.

Aim: To review in utero transfers (IUT), their antenatal optimisation strategies including predictive testing and outcomes in a large region in the UK.

Methods: All IUT requests received by regional transport service in the UK, over a 12-month period, were reviewed gathering information on gestation at referral, antenatal corticosteroid use, tocolysis, magnesium sulphate, predictive test use (and result if applicable) and delivery outcome at 48 h. The reason for transfer was also collated to determine capacity issues.

Serum HCoV-spike specific antibodies do not protect against subsequent SARS-CoV-2 infection in children and adolescents.

SARS-CoV-2 infections in children are generally asymptomatic or mild and rarely progress to severe disease and hospitalization. Why this is so remains unclear. Here we explore the potential for protection due to pre-existing cross-reactive seasonal coronavirus antibodies and compare the rate of antibody decline for nucleocapsid and spike protein in serum and oral fluid against SARS-CoV-2 within the pediatric population.

How Much Does Paediatric Femoral Lengthening Cost? A Cost Comparison between Magnetic Lengthening Nails and External Fixators.

Aim: Motorised intramedullary lengthening nails are considered more expensive than external fixators for limb lengthening. This research aims to compare the cost of femoral lengthening in children using the PRECICE magnetic lengthening nail with external fixation.

Methods: Retrospective analysis of 50 children who underwent femoral lengthening.

Digital technology for early identification of exacerbations in people with cystic fibrosis.

Background: Cystic fibrosis (CF) is a life-limiting genetic condition affecting various organ systems including the gastrointestinal tract, endocrine system and especially the respiratory tract. Pulmonary exacerbations in CF result in increased symptoms, an acceleration in the rate of lung decline and an increased need for treatment. Early detection of infections or clinical worsening provides an opportunity for proactive treatment that may affect clinical outcomes.

Alterations of mucosa-attached microbiome and epithelial cell numbers in the cystic fibrosis small intestine with implications for intestinal disease.

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Defective CFTR leads to accumulation of dehydrated viscous mucus within the small intestine, luminal acidification and altered intestinal motility, resulting in blockage. These changes promote gut microbial dysbiosis, adversely influencing the normal proliferation and differentiation of intestinal epithelial cells.

Applicability, efficacy, and safety of over-the-scope clips in children.

Background And Aims: Over-the-scope clips (OTSCs) are now becoming popular in endoscopy performed in adults for indications such as acute nonvariceal GI bleeding, anastomotic bleeding, and for closure of gastrocutaneous and postgastrostomy fistulae. Varied sizes of clip are available, but even the smallest, 8.5 to 9.

Stigma from medication use: an under recognised burden of care.

Unlabelled: Children with respiratory diseases take treatments for the self-management of symptoms and to maintain disease control. Often, these treatments need to be taken in social environments like school. Respiratory treatments can foster a feeling of difference and stigmatisation, which negatively impact on the quality of life and adherence to treatment.

Routine use of the Bath Adolescent Pain Questionnaire in a paediatric pain clinic.

Introduction: Assessment of outcomes from health interventions are of increasing importance, primarily to identify effective and safe treatment, but also to justify funding decisions. The Bath Adolescent Pain Questionnaire (BAPQ) is a self-report questionnaire, validated in 11-18 year olds, assessing the impact of pain in multiple domains of adolescent life. The similarly validated Bath Adolescent Pain Questionnaire for Parents (BAPQ-P) uses the same domains as the BAPQ, assessing the functioning and development of the adolescent from the parents' perspective.

Pelvic Support Hip Reconstruction with Internal Devices: An Alternative to Ilizarov Hip Reconstruction.

Aim And Objective: Ilizarov hip reconstruction (IHR) is a traditional method of salvaging chronic adolescent problem hips but faces practical problems from external fixators leading to reduced compliance. We present the same reconstruction utilising only internal devices with a modification in technique and aim to review early results.

Materials And Methods: We retrospectively evaluated eight patients between 2014 and 2017 with chronic painful hips treated by a two-stage reconstruction; stage 1 included femoral head resection and pelvic support osteotomy using double plating, while stage 2 comprised distal femoral osteotomy avoiding varus followed by insertion of retrograde magnetic nail for postoperative lengthening.

Outcomes of distally un-threaded screw fixation of slipped capital femoral epiphysis at skeletal maturity: a matched cohort study.

The most common treatment for slipped capital femoral epiphysis worldwide is in situ fixation with a threaded screw. Un-threaded screws are designed to prevent slip progression without hindering residual growth of the proximal femur. This study aimed to compare growth, remodelling and long-term outcomes after fixation with un-threaded screws and a matched cohort of patients treated with a standard screw.

Vitamin E supplementation in people with cystic fibrosis.

Background: People with cystic fibrosis are at an increased risk of fat-soluble vitamin deficiency, including vitamin E. Vitamin E deficiency can cause a host of conditions such as haemolytic anaemia, cerebellar ataxia and cognitive difficulties. Vitamin E supplementation is widely recommended for people with cystic fibrosis and aims to ameliorate this deficiency.

Correction of Bowleg Deformity in Achondroplasia through Combined Bony Realignment and Lateral Collateral Ligament Tightening.

Introduction: Achondroplasia is one of the most common osteochondrodysplasias with an incidence of 1 in 26,000 live births. Bowing of lower limbs can cause significant morbidity in this population. The use of the Ilizarov external fixator to tighten collateral ligaments of the knee in children has not been reported in the literature.

Classification of sleep-related sudden unexpected death in infancy: A national survey.

Aim: To identify how British Child Death Overview Panels (CDOPs) and paediatric pathologists classify cause of death for sleep-related Sudden Unexpected Death in Infancy (SUDI). To determine compliance with national requirements for SUDI investigation.

Methods: Electronic survey of CDOPs and pathologists using three vignettes of SUDI cases illustrating: accidental asphyxia, typical Sudden Infant Death Syndrome (SIDS) and SIDS with co-sleeping.

Recurrent sudden unexpected death in infancy: a case series of sibling deaths.

Objectives: To determine the rate of sudden unexpected death in infancy (SUDI) for infants born after a previous SUDI in the same family, and to establish the causes of death and the frequency of child protection concerns in families with recurrent SUDI.

Design: Observational study using clinical case records.

Setting: The UK's Care of Next Infant (CONI) programme, which provides additional care to families who have experienced SUDI with their subsequent children.

Reducing pediatric exposure to environmental tobacco smoke: The effects of pediatric exposure to environmental tobacco smoke and the role of pediatric perioperative care.

Exposure to environmental tobacco smoke (ETS) has deleterious effects on a child's general health and their perioperative risk; specifically, it doubles a child's perioperative risk of adverse respiratory events, particularly laryngospasm. It increases the risk of sudden infant death syndrome, bacterial meningitis, middle ear infection, asthma, and lower respiratory tract infection. The preoperative assessment of children presenting for procedures under general anesthesia is an opportune moment to screen for exposure to ETS and give information about the risks and cessation support (if applicable).

Non classic presentations of a genetic mutation typically associated with transient neonatal diabetes.

Summary: This case report describes a family pedigree of a mother and her children with an E227K mutation in the KCNJ11 gene. People with this particular gene mutation typically present with transient neonatal diabetes; with more than half the cohort relapsing into permanent diabetes in adolescence or early adulthood. However, the mother developed diabetes as an adolescent and thus was initially diagnosed as having Type 1 Diabetes.

Validity of the International Prostate Symptoms Score in predicting urinary retention after joint replacement.

Postoperative urinary retention necessitating catheterization after major lower limb arthroplasty surgery adds to the patients' postoperative discomfort and increases the risk of urinary tract infection with potential risk of transient bacteraemia and seeding of infection to prosthetic joints. Preoperative evaluation of patients with lower urinary tract symptoms may help to identify at-risk patients and the International Prostate Symptoms Score (IPSS) has been used as a screening tool to quantify the severity of symptoms in males. A prospective cohort of 303 patients undergoing total hip or knee arthroplasty was evaluated using the IPSS.

Disrupted apical exocytosis of cargo vesicles causes enteropathy in FHL5 patients with Munc18-2 mutations.

Familial hemophagocytic lymphohistiocytosis 5 (FHL5) is an autosomal recessive disease caused by mutations in STXBP2, coding for Munc18-2, which is required for SNARE-mediated membrane fusion. FHL5 causes hematologic and gastrointestinal symptoms characterized by chronic enteropathy that is reminiscent of microvillus inclusion disease (MVID). However, the molecular pathophysiology of FHL5-associated diarrhea is poorly understood.

Vitamin E supplementation in people with cystic fibrosis.

Background: People with cystic fibrosis are at an increased risk of fat-soluble vitamin deficiency including vitamin E. Vitamin E deficiency can cause a host of conditions such as haemolytic anaemia, cerebellar ataxia and cognitive difficulties. Vitamin E supplementation is widely recommended in cystic fibrosis and aims to ameliorate this deficiency.

Candidate gene variants of the immune system and sudden infant death syndrome.

Background: Sudden infant death syndrome (SIDS) causes early infant death with an incidence between 0.5 and 2.5 cases among 1000 live births.

Screening for spinal stenosis in achondroplastic patients undergoing limb lengthening.

The need for a screening programme for spinal stenosis in children with achondroplasia undergoing limb lengthening was identified in a tertiary limb reconstruction service. The aim of this study was to evaluate whether screening would identify the 'at risk' group. A total of 26 achondroplastic patients underwent our screening programme.