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This article explores the historical origins and international perspectives of two medical conditions named after Freiherr von Münchhausen. Munchausen syndrome is a mental health disorder where individuals feign physical or psychological illness for attention, while Munchausen syndrome by proxy refers to a type of child abuse where caregivers fabricate or induce illness in their child. The article discusses the history of these syndromes and provides information on their diagnosis, risk factors, and management. It also highlights the importance of distinguishing these conditions from malingering and provides insights into the legal and psychological aspects of Munchausen syndrome by proxy. [Extracted from the article]

Fibre is an essential nutrient in the human diet that is crucial for human health. It provides a range of functional benefits, including stool bulking, and physiological benefits through fermentation of diverse fibre types by the gut microbiome including cholesterol lowering, glycaemic control and weight control. The by-products of the fermentation of fibre in the gut confer health benefits that extend beyond the gut to the immune system and organs such as the liver, kidneys and the brain. A lack of fibre in the diet has been associated with several disorders in children including constipation, irritable bowel syndrome, allergies and immune-related disorders. In paediatric practice, concerns exist over tolerance of dietary fibre which may lead to unnecessary restrictions, especially for children receiving nutritional support. One reason for this may be the terminology which has historically been used. Fibre is often described in terms of its physico-chemical properties (solubility, viscosity), rather than its physiological effects/functionality (fermentability, bulking effects). To describe fibre in these latter terms represents more clearly the important role it plays. Most international guidelines recommend a daily quantity of fibre, failing to mention the quality aspect of the fibre required for health. Here we consider the evidence base for the current recommendations for daily fibre intakes for healthy children, those requiring nutritional support and those with functional gastrointestinal disorders. We also consider the importance of the gut microbiome and the role of fibre in maintaining gut microbial health and its role in health beyond the gut. [ABSTRACT FROM AUTHOR]

Background: Early recognition of children at risk of severe respiratory syncytial virus (RSV) lower respiratory tract infection is important as it informs management decisions. We aimed to evaluate factors associated with severe disease among young children hospitalised with RSV infection.Methods: We conducted a retrospective cohort study of all children <2 years of age hospitalised for RSV lower respiratory tract infection at a single tertiary paediatric hospital over three RSV seasons (January 2017-December 2019). We classified children as having 'moderate' or 'severe' disease based on the level of respiratory intervention and used univariable and multivariable regression models to determine factors associated with severe disease.Results: Of 970 hospitalised children, 386 (40%) were classified as having 'severe' and 584 (60%) as having 'moderate' RSV disease. On multivariable analyses, age <2 months (OR: 2.3, 95% CI 1.6 to 3.3, p<0.0001), prematurity (OR: 1.6, 95% CI 1.1 to 2.4, p=0.02) and RSV-parainfluenza virus type 3 (PIV3) codetection (OR: 2.6, 95% CI 1.05 to 6.5, p=0.04) were independently associated with severe disease.Conclusion: Younger age, prematurity and PIV3 codetection were associated with severe RSV disease in children <2 years of age hospitalised with RSV infection. The association between PIV3 and severe RSV disease is a novel finding and warrants further investigation. [ABSTRACT FROM AUTHOR]

Knowledge of thrombosis in children with SARS-CoV-2 is scarce. In this multicentre national cohort of children with SARS-CoV-2 involving 49 hospitals, 4 patients out of 537 infected children developed a thrombotic complication (prevalence of 0.7% (95% CI: 0.2% to 1.9%) out of the global cohort and 1.1% (95% CI: 0.3% to 2.8%) out of the hospitalised patients). We describe their characteristics and review other published paediatric cases. Three out of the four patients were adolescent girls, and only two cases had significant thrombotic risk factors. In this paediatric cohort, D-dimer value was not specific enough to predict thrombotic complications. Adolescence and previous thrombotic risk factors may be considered when initiating anticoagulant prophylaxis on children with SARS-CoV-2 disease (COVID-19). [ABSTRACT FROM AUTHOR]

Objective: This study describes core outcomes of Hirschsprung's disease (HD) in a UK-wide cohort of primary school-aged children.Design: A prospective cohort study conducted from 1 October 2010 to 30 September 2012. Outcomes data were collected from parents and clinicians when children were 5-8 years of age, and combined with data collected at birth, and 28 days and 1 year post diagnosis.Setting: All 28 UK and Irish paediatric surgical centres.Participants: Children with histologically proven HD diagnosed at <6 months of age.Main Outcome Measures: NETS1HD core outcomes.Results: Data were returned for 239 (78%) of 305 children. Twelve children (5%) died prior to 5 years of age.Of the 227 surviving children, 30 (13%) had a stoma and 21 (9%) were incontinent of urine. Of the 197 children without a stoma, 155 (79%) maintained bowel movements without enemas/washouts, while 124 (63%) reported faecal incontinence. Of the 214 surviving children who had undergone a pull-through operation, 95 (44%) underwent ≥1 unplanned reoperation. 89 unplanned reoperations (27%) were major/complex.Of the 83 children with returned PedsQL scores, 37 (49%) had quality of life scores, and 31 (42%) had psychological well-being scores, that were ≥1 SD lower than the reference population mean for children without HD.Conclusion: This study gives a realistic picture of population outcomes of HD in primary school-aged children in the UK/Ireland. The high rates of faecal incontinence, unplanned procedures and low quality of life scores are sobering. Ensuring clinicians address the bladder, bowel and psychological problems experienced by children should be a priority. [ABSTRACT FROM AUTHOR]

Liver involvement in sickle cell disease (SCD) is often referred to as sickle cell hepatopathy (SCH) and is a complication of SCD which may be associated with significant mortality. This review is based on a round-table workshop between paediatric and adult hepatologists and haematologists and review of the literature. The discussion was prompted by the lack of substantial data and guidance in managing these sometimes very challenging cases. This review provides a structured approach for the diagnosis and management of SCH in children and young adults. The term SCH describes any hepatobiliary dysfunction in the context of SCD. Diagnosis and management of biliary complications, acute hepatic crisis, acute hepatic sequestration and other manifestations of SCH are discussed, as well as the role of liver transplantation and haemopoietic stem cell transplantation in the management of SCH. [ABSTRACT FROM AUTHOR]

Objective: To describe neurodevelopment and follow-up services in preschool children with heart disease (HD).Design: Secondary analysis of a prospectively collected multicentre dataset.Setting: Three London tertiary cardiac centres.Patients: Preschool children<5 years of age: both inpatients and outpatients.Methods: We analysed results of Mullen Scales of Early Learning (MSEL) and parental report of follow-up services in a representative convenience sample evaluated between January 2014 and July 2015 within a previous study.Results: Of 971 preschool children: 577 (59.4%) had ≥1 heart operation, 236 (24.3%) had a known diagnosis linked to developmental delay (DD) ('known group') and 130 (13.4%) had history of clinical event linked to DD. On MSEL assessment, 643 (66.2%) had normal development, 181 (18.6%) had borderline scores and 147 (15.1%) had scores indicative of DD. Of 971 children, 609 (62.7%) were not receiving follow-up linked to child development and were more likely to be under these services with a known group diagnosis, history of clinical event linked to DD and DD (defined by MSEL). Of 236 in known group, parents of 77 (32.6%) and of 48 children not in a known group but with DD 29 (60.4%), reported no child development related follow-up. DD defined by MSEL assessment was more likely with a known group and older age at assessment.Conclusions: Our findings indicate that a 'structured neurodevelopmental follow-up pathway' in preschool children with HD should be considered for development and evaluation as children get older, with particular focus on those at higher risk. [ABSTRACT FROM AUTHOR]

Background: The prevalence of and risk factors for neurological problems in childhood coeliac disease (CD) are unclear.Methods: We performed a cross-sectional, community-based audit of CD in children diagnosed from January 2010 to December 2016 in Lothian.Results: 79 (28%) of 284 children with CD (201, 70.8% female) (mean age 8.3 years, range of 1-16) had neurological problems. Fifteen (5.3%) had headaches/migraine, 10 (3.5%) anxiety, 8 (2.8%) motor/co-ordination problems / ataxia (there were no patients with ataxia), 7 (2.5%) had behavioural issues, 5 (1.8 %) with ASD, 5 (1.8%) low mood, 4 (1.4%) ADD/ ADHD, 3 (1.1%) seizures and 2 (0.7%) had neuropathy. Neurological problems were more common with later age at CD diagnosis (OR 1.07, 95% CI 1.01 to 1.14) and male gender (OR 1.69, 95% CI 0.96 to 2.95).Conclusion: Prevalence of neurological problems in children with CD in Lothian is lower than published adult CD studies and similar or lower to the reported prevalence in the general childhood population. [ABSTRACT FROM AUTHOR]

Non-alcoholic fatty liver disease (NAFLD) in children and adolescents has an estimated prevalence of 36.1% in the context of obesity. This figure is anticipated to increase in conjunction with the global obesity epidemic. Worryingly, NAFLD in childhood persisting into adulthood is likely to be harmful, contributing to significant hepatic and extrahepatic morbidities. Early disease detection is required, although the optimum timing, frequency and mode of screening remains undetermined. While the efficacy of several medications, antioxidants, fatty acid supplements and probiotics has been investigated in children, healthy eating and physical activity remain the only prevention and treatment strategies for paediatric NAFLD. This short review discusses the epidemiology, diagnosis, pathogenesis and management of NAFLD in childhood obesity. [ABSTRACT FROM AUTHOR]

The centrally coordinated response that controlled the polio epidemics of the 1950s through immunisation led to the development of a national immunisation strategy in the UK and the formation of the Joint Committee on Vaccination and Immunisation (JCVI) in 1963, which oversees the immunisation programme and advises the UK Department of Health on new vaccine introductions. As a result of technological advances in vaccine development and scientific advances in immunology and microbiology over the 56 years since then, and the formation of a comprehensive public health surveillance system for vaccine-preventable disease, the National Health Service immunisation programme now covers 18 serious diseases of childhood, with an astonishing impact on child health. Here we consider the formation of the JCVI and the development of the national immunisation programme and review the introduction of vaccines over the past half century to defend public health. [ABSTRACT FROM AUTHOR]

Objective: We aimed to measure the prevalence and incidence of latent tuberculosis infection (LTBI) and tuberculosis (TB) disease in children in close contact with patients with drug-resistant TB (DR-TB) in a country with high DR-TB prevalence.Design and Setting: This is a prospective cohort study of paediatric contacts of adult patients with pulmonary DR-TB in Armenia. Children were screened using tuberculin skin test, interferon-gamma release assay and chest X-ray at the initial consultation, and were reassessed every 3-6 months for a period of 24 months. Children did not receive preventive treatment.Main Outcome Measures: Prevalence and incidence of LTBI and TB disease; factors associated with prevalent LTBI.Results: At initial evaluation, 3 of the 150 children included were diagnosed with TB disease (2.0%). The prevalence of LTBI was 58.7%. The incidence of LTBI was 19.9 per 100 children per year, and was especially high during the first 6 months of follow-up (33.3 per 100 children per year). No additional cases with incident disease were diagnosed during follow-up. After adjustment, prevalent LTBI was significantly associated with the child's age, sleeping in the same house, higher household density, the index case's age, positive smear result and presence of lung cavities.Conclusions: Children in close contact with patients with DR-TB or in contact with very contagious patients had an increased risk of prevalent LTBI. Although none of the children developed TB disease during a 2-year follow-up period, screening for symptoms of TB disease, based on the prevalence of disease at recruitment, together with follow-up and repeated testing of non-infected contacts, is highly recommended in paediatric contacts of patients with DR-TB. [ABSTRACT FROM AUTHOR]

Objective: We aimed to compare the severity of coronary artery abnormalities in Kawasaki disease between infants and older children.Methods: We retrospectively reviewed and compared coronary artery dilation and aneurysm severity in infants <1 year of age with Kawasaki disease at our centre over a 10-year period with that observed in children ≥1 year of age in the Pediatric Heart Network Trial of Pulse Steroid Therapy in Kawasaki Disease. Coronary artery abnormalities were defined by z-scores according to American Heart Association guidelines.Results: Of the 93 infants identified during the study period, 80 were treated with intravenous gamma globulin within the first 10 days of illness and were included for comparison to 170 children ≥1 year of age treated in the same time frame from the Pediatric Heart Network public database. The mean maximum z-score was significantly higher in infants compared with older children (3.37 vs 2.07, p<0.001). A higher incidence of medium and giant aneurysms was observed in infants compared with children ≥1 year of age (11% vs 3% for medium aneurysms, p=0.015; 8% vs <1% for giant aneurysms, p=0.005).Conclusions: Infants with Kawasaki disease have more severe coronary artery dilation compared with older children, and a higher prevalence of medium and giant aneurysms. Because adverse outcomes are closely linked to the maximal coronary artery diameter in Kawasaki disease, patients diagnosed as infants require very close long-term monitoring for cardiac complications. [ABSTRACT FROM AUTHOR]

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.Participants: Between May 1997 and October 2017, a total of 2050 cases meeting PIND criteria had been notified and investigated.Results: Six children had vCJD. 1819 children had other diagnoses, made in 12 cases by antemortem brain biopsy and in 15 by postmortem investigations. 225 children were undiagnosed: only 3 had antemortem brain biopsies and only 14 of the 108 who died were known to have had autopsies; postmortem neuropathological studies were carried out in just 10% (11/108) and only two had prion protein staining of brain tissue. Of the undiagnosed cases 43% were known to come from Asian British families.Conclusions: Most of the notified children had a diagnosis other than vCJD to explain their neurological deterioration. None of the undiagnosed cases had the clinical phenotype of vCJD but brain tissue was rarely studied to exclude vCJD. Clinical surveillance via the PIND Study remains the only practical means of searching for vCJD in UK children. [ABSTRACT FROM AUTHOR]

Background: As survival improves in the congenital heart disease (CHD) population, health-related quality of life (HRQOL) outcomes become increasingly important. While surgery improves survival, poor HRQOL occurs postoperatively and cardiac-related HRQOL outcomes are rarely reported.Objective: To conduct a systematic review and meta-analyses of general and cardiac-related HRQOL in CHD surgical children and young adults.Method: Medline, CINAHL and EMBASE were searched. Quantitative designs with a minimum of 80% CHD surgical patients and mean age ≤18 years compared with healthy controls were included in the review. Data were analysed in RevMan V.5.3 using a random effects model.Outcome Measures: General and cardiac-related HRQOL.Results: Studies (n=20) were conducted in high-income countries and included 3808 patients plus 2951 parental reports of patients. HRQOL was worse in postoperative patients with CHD versus healthy controls in all domains with the largest difference seen for physical function (standard mean difference (SMD) of -0.56, 95% CI -0.82 to -0.30). Cardiac-related HRQOL was worse in complex compared with simple CHD with the largest SMD (-0.60, 95% CI -0.80 to -0.40) for symptoms. Heterogeneity ranged from 0% to 90%.Conclusions: CHD surgical patients have substantially worse HRQOL compared with age-matched healthy controls. Strategies should focus on improving HRQOL in this subgroup. Results may not be applicable to low/middle-income countries given the dearth of relevant research. [ABSTRACT FROM AUTHOR]

Objective: Myotonic dystrophy type 1 (DM1) is an autosomal-dominant neuromuscular disease with variable severity affecting all ages; however, current care guidelines are adult-focused. The objective of the present study was to profile DM1 in childhood and propose a framework to guide paediatric-focused management.Design, Setting and Patients: 40 children with DM1 (mean age 12.8 years; range 2-19) were studied retrospectively for a total of 513 follow-up years at Sydney Children's Hospital. 143 clinical parameters were recorded.Results: The clinical spectrum of disease in childhood differs from adults, with congenital myotonic dystrophy (CDM1) having more severe health issues than childhood-onset/juvenile patients (JDM1). Substantial difficulties with intellectual (CDM1 25/26 96.2%; JDM1 9/10, 90.0%), fine motor (CDM1 23/30, 76.6%; JDM1 6/10, 60.0%), gastrointestinal (CDM1 17/30, 70.0%; JDM1 3/10, 30.0%) and neuromuscular function (CDM1 30/30, 100.0%; JDM1 25/30, 83.3%) were evident.Conclusion: The health consequences of DM1 in childhood are diverse, highlighting the need for paediatric multidisciplinary management approaches that encompass key areas of cognition, musculoskeletal, gastrointestinal, respiratory, cardiac and sleep issues. [ABSTRACT FROM AUTHOR]

Objective: To evaluate research priority setting approaches in childhood chronic diseases and to describe the priorities of stakeholders including patients, caregivers/families and health professionals.Design: We conducted a systematic review of MEDLINE, Embase, PsycINFO and CINAHL from inception to 16 October 2016. Studies that elicited stakeholder priorities for paediatric chronic disease research were eligible for inclusion. Data on the prioritisation process were extracted using an appraisal checklist. Generated priorities were collated into common topic areas.Results: We identified 83 studies (n=15 722). Twenty (24%) studies involved parents/caregivers and four (5%) children. The top three health areas were cancer (11%), neurology (8%) and endocrine/metabolism (8%). Priority topic areas were treatment (78%), disease trajectory (48%), quality of life/psychosocial impact (48%), disease onset/prevention (43%), knowledge/self-management (33%), prevalence (30%), diagnostic methods (28%), access to healthcare (25%) and transition to adulthood (12%). The methods included workshops, Delphi techniques, surveys and focus groups/interviews. Specific methods for collecting and prioritising research topics were described in only 60% of studies. Most reviewed studies were conducted in high-income nations.Conclusions: Research priority setting activities in paediatric chronic disease cover many discipline areas and have elicited a broad range of topics. However, child/caregiver involvement is uncommon, and the methods often lack clarity. A systematic and explicit process that involves patients and families in partnership may help to inform a more patient and family-relevant research agenda in paediatric chronic disease. [ABSTRACT FROM AUTHOR]

Objective: Compare trends in coronary artery (CA) abnormality diagnoses to trends in adverse cardiac outcomes among American children with Kawasaki disease (KD) to assess the fit of detection of CA abnormalities to an established model of overdiagnosis.Design: Multicenter retrospective cohort.Setting: 48 US children's hospitals in the Paediatric Health Information System database.Participants: Children <18 years receiving care for KD between 2000 and 2014.Main Outcome Measures: The main outcomes were rates of CA abnormality diagnoses and adverse cardiac outcomes, measured during a child's incident KD visit and longitudinally at all subsequent visits to the same hospital, through December 2016. CA abnormalities were considered severe if long-term anticoagulation other than aspirin was prescribed. Trends were tested using mixed effects logistic regression, adjusting for patient demographics.Results: Among 17 809 children treated for KD, a CA abnormality was diagnosed in 1435 children (8%), including 1117 considered non-severe and 318 severe. The rate of non-severe CA abnormality diagnoses increased from 45 per 1000 patients with KD in 2000 to 81 per 1000 patients with KD in 2014, representing an adjusted 2.3-fold increased odds (95% CI 1.8 to 3.0) of diagnosis. There was no significant change in diagnoses of severe CA abnormalities. Adverse cardiac outcomes were stable over the study period at 19 per 1000 patients with KD (P=0.24 for trend).Conclusions: The rising rate of detection of non-severe CA abnormalities accompanied by an unchanging rate of adverse cardiac outcomes among American children with KD fits an overdiagnosis pattern. [ABSTRACT FROM AUTHOR]

Objective: In an attempt to improve the diagnosis of sepsis in children, diagnostic aids have concentrated on clinical features that suggest that sepsis is present. Clinicians need to be able to clinically rule out sepsis as well as rule it in. Little is known about which features are consistent with wellness and/or absence of sepsis. Guidelines are therefore likely to improve sensitivity without preserving specificity. We aimed to gather expert opinion on which (if any) features would make clinicians consider a child to be unlikely to have sepsis.Design: We undertook a modified two-round international Delphi study, where clinicians were asked for features they believed were indicators of wellness in an ill child.Participants: One hundred and ninety-five clinicians (predominantly physicians) who routinely assessed unwell children and had been doing so for most of their careers.Results: Over 90% of respondents rated age-appropriate verbalisation, playing, smiling and activity as reassuring that a child was unlikely to have sepsis. Eating, spontaneous interaction and normal movement were also agreed to be reassuring by over 70% of participants. Consolability and showing fear of the clinician were not felt to be adequately reassuring. There was wide range of opinion on how reassuring the use of an electronic device was thought to be.Conclusions: This study confirms that physicians are reassured by specific behaviours in ill children, and provides a framework which may be used to help guide the assessment of the unwell child. Validation of individual features could lead to improved specificity of diagnostic aids for diagnosing sepsis. [ABSTRACT FROM AUTHOR]

Objective: To examine in a community-based cohort: (1) the prevalence of chronic tic disorder (CTD) in children with attention-deficit/hyperactivity disorder (ADHD) compared with non-ADHD controls at ages 7 and 10; and (2) the additional psychiatric and functional burden of CTD in children with ADHD.Methods: Children aged 6-8 years with ADHD (n=179) and controls (n=212) were recruited through 43 Victorian schools using parent and teacher screening surveys (Conners 3 ADHD Index), followed by case confirmation (Diagnostic Interview Schedule for Children-IV (DISC-IV)). CTD was identified using the DISC-IV categories chronic motor tic disorder, chronic vocal tic disorder or Tourette syndrome at baseline and 36-month follow-up. Internalising and externalising disorders, social functioning, academic performance and quality of life were also measured. Tests of proportions and independent t-tests were used to compare the ADHD+CTD group with sex-matched ADHD alone children.Results: Compared with controls, children with ADHD were 4.1 (95% CI 1.1 to 14.1) times more likely to have CTD at age 7, and 5.9 (95% CI 1.6 to 17.9) times more likely at age 10. Children with ADHD+CTD experienced higher rates of internalising disorders and peer problems, and poorer quality of life than those with ADHD alone.Conclusions: CTD prevalence is higher in children with ADHD compared with controls, and confers substantial additional psychiatric and functional burden. Clinicians need to consider CTD in both the initial assessment and ongoing management of children with ADHD, and address both the symptoms and the associated impairments. [ABSTRACT FROM AUTHOR]

Background: We sought to determine clinical variables in children tested for suspected pulmonary embolism (PE) that predict PE+ outcome for the development of paediatric PE prediction rule.Methods: Data were collected by query of a laboratory database for D-dimer from January 2004 to December 2014 for a large multicentre hospital system and the radiology database for pulmonary vascular imaging in children aged 5-17. Using explicit, predefined methods, trained abstractors, determined if D-dimer was sent in the evaluation of PE and then recorded predictor data which was tested for association with PE+ outcome using univariate techniques.Results: D-dimer was ordered in 526 children for clinical suspicion of PE. Thirty-four of 526 were PE+ (6.4%, 95% CI 4.3% to 8.7%). The radiology database identified 17 additional patients with PE (n=51 PE+ total). Children evaluated for PE were primarily in the ED setting (80%), teenagers (88%) and 2:1 female:male. Children with PE had higher mean heart and higher respiratory rate and a lower pulse oximetry and haemoglobin concentration. On univariate analysis, five conditions were more frequent in PE+ compared with no PE: surgery, central line, limb immobility, prior PE or deep vein thrombosis and cancer.Conclusions: The rate of PE diagnosis in children with D-dimer was 6.4%, similar to that seen in adults; most children with PE are over 13 years and had clinical predictors known to increase probability of PE in symptomatic adults. Future studies should use these criteria to develop a clinical decision rule for PE in children. [ABSTRACT FROM AUTHOR]

Optimising the dosing of medicines for neonates and children remains a challenge. The importance of pharmacokinetic (PK) and pharmacodynamic (PD) research is recognised both in medicines regulation and paediatric clinical pharmacology, yet there remain barriers to undertaking high-quality PK and PD studies. While these studies are essential in understanding the dose-concentration-effect relationship and should underpin dosing recommendations, this review examines how challenges affecting the design and conduct of paediatric pharmacological studies can be overcome using targeted pharmacometric strategies. Model-based approaches confer benefits at all stages of the drug life-cycle, from identifying the first dose to be used in children, to clinical trial design, and optimising the dosing regimens of older, off-patent medications. To benefit patients, strategies to ensure that new PK, PD and trial data are incorporated into evidence-based dosing recommendations are needed. This review summarises practical strategies to address current challenges, particularly the use of model-based (pharmacometric) approaches in study design and analysis. Recommendations for practice and directions for future paediatric pharmacological research are given, based on current literature and our joint international experience. Success of PK research in children requires a robust infrastructure, with sustainable funding mechanisms at its core, supported by political and regulatory initiatives, and international collaborations. There is a unique opportunity to advance paediatric medicines research at an unprecedented pace, bringing the age of evidence-based paediatric pharmacotherapy into sight. [ABSTRACT FROM AUTHOR]

While maternal and perinatal mortality auditing has been strongly promoted by the World Health Organization (WHO), there has been very limited promotion or evaluation of child death auditing in low/middle-income settings. In 2017, a standardised child death review process was introduced in the paediatric department of the National Hospital in Honiara, Solomon Islands. We evaluated the process and outcomes of child death reviews. The child death auditing process was assessed through systematic observations made at each of the weekly meetings using the following standards for evaluation: (1) adapted WHO tools for paediatric auditing; (2) the five stages of the audit cycle; (3) published principles of paediatric audit; and (4) WHO and Solomon Islands national clinical standards of Hospital Care for Children. Thirty-three child death review meetings were conducted over 6 months, reviewing 66 neonatal and child deaths. Some areas of the process were satisfactory and other areas were identified for improvement. The latter included use of a more systematic classification of causes of death, inclusion of social risk factors and community problems in the modifiable factors and more follow-up with implementation of action plans. Areas for improvement were in communication, clinical assessment and treatment, availability of laboratory tests, antenatal clinic attendance and equipment for high dependency neonatal and paediatric care. Many of the changes recommended by audit require a quality improvement team to implement. Child death auditing can be done in resource-limited settings and yield useful information of gaps which are linked to preventable deaths; however, using the data to produce meaningful changes in practice is the greatest challenge. Audit is an iterative and evolving process that needs a structure, tools, evaluation, and needs to be embedded in the culture of a hospital as part of overall quality improvement, and requires a quality improvement team to follow-up and implement action plans. [ABSTRACT FROM AUTHOR]

Background: Congenital lymphoedema is a lifelong condition that has detrimental physical and psychosocial outcomes for young patients and burdensome treatment responsibilities that may hamper patients' motivation for self-management. There is limited research from the perspective of young people with primary lymphoedema. We aimed to describe the experiences and views of children and adolescents with lymphoedema to inform patient-centred practice.Methods: Twenty patients (aged 8-21 years) with primary lymphoedema were purposively sampled from two paediatric clinics in Sydney, Australia, to participate in a semistructured interview. The transcripts were analysed thematically.Results: We identified six themes: reinforcing abnormality (damaging self-esteem, self-consciousness, frustrating restrictions and isolation); negotiating uncertainties (fearing condition worsening, deprioritised and sidelined, questioning cause and permanence, widespread unawareness); vulnerability and caution (avoiding pain and discomfort, preventing severe and permanent consequences, depending on permission, limiting goals and aspirations); disruptive transition (resisting change, losing progress and support, avoiding treatment costs); developing resilience (focusing on the positives, embracing individuality, recalibrating normality, prioritising coping) and taking responsibility (individualising treatment, needing support, external pressure and motivation, sticking to a routine, seeking independence).Conclusion: Children and adolescents learn to adjust to the daily demands of lymphoedema management by individualising and accepting their treatment, but many continue to struggle with their self-esteem and lifestyle restrictions. Strategies are needed to empower young patients to advocate for themselves during their transition to adult care. Treatment plans that minimise social restrictions, address emotional consequences and incorporate patients' preferences could improve adherence, satisfaction and outcomes. [ABSTRACT FROM AUTHOR]

Objective: To determine the prevalence of invasive bacterial infections (IBI, pathogenic bacteria in blood or cerebrospinal fluid) in infants less than 90 days old with fever without a source related to the presence or absence of fever on arrival to the emergency department (ED).Design: Prospective registry-based cohort study.Setting: Paediatric ED of a tertiary teaching hospital.Patients: We included infants less than 90 days old with a history of fever evaluated in the ED from 2003 to 2016.Main Outcomes and Measures: The prevalence of IBI in patients with a history of fever who were febrile and afebrile on arrival to the ED.Results: We included 2470 infants: 678 afebrile and 1792 febrile when evaluated in the ED. Fifty-nine (2.4%) were diagnosed with an IBI (bacteraemia 46, meningitis 7 and sepsis 6): 16 in the group of afebrile infants with a history of fever (2.4%, 95% CI 1.4 to 3.8 vs 43 in the febrile group, 2.4%, 95% CI 1.8 to 3.2). Of the 16 afebrile infants with a history of fever diagnosed with an IBI, 14 were well appearing. The rate of non-IBI (pathogenic bacteria in urine or stools) was similar in both groups (15.5% and 16.7%).Conclusions: The prevalence of IBI in infants ≤90 days with a history of fever is similar regardless of the presence of fever on the arrival at the ED. The approach to infants with a history of fever who are afebrile in the ED should not differ from that recommended for infants who are febrile in the ED. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the diagnosis and management of reflux and gastro-oesophageal reflux disease (GORD) in infants aged <1 year presenting to general practitioners (GPs).Design, Setting and Participants: A nationally representative, prospective, cross-sectional survey of GP activity in Australia, 2006-2016 (Bettering the Evaluation And Care of Health Study). Annually, a random sample of around 1000 GPs recorded details for 100 consecutive visits with consenting, unidentified patients.Outcome Measures: Diagnoses of reflux and GORD and their management including prescribing of acid-suppressant medicines (proton pump inhibitors (PPIs) and histamine receptor antagonists (H2RAs)) and counselling, advice or education.Results: Of all infants' visits, 512 (2.7%) included a diagnosis of reflux (n=413, 2.2%) or GORD (n=99, 0.5%). From 2006 to 2016, diagnostic rates decreased for reflux and increased for GORD. Prescribing of acid suppressants occurred in 43.6% visits for reflux and 48.5% visits for GORD, similar to rates of counselling, advice or education (reflux: 38.5%, GORD: 43.4% of visits). Prescribing of PPIs increased (statistically significant only for visits for reflux), while prescribing of H2RAs decreased.Conclusions: Overprescribing of acid suppressants to infants may be occurring. In infants, acid-suppressant medicines are no better than placebo and may have significant negative side effects; however, guidelines are inconsistent. Clear, concise and consistent guidance is needed. GPs and parents need to understand what is normal and limitations of medical therapy. We need a greater understanding of the influences on GP prescribing practices, of parents' knowledge and attitudes and of the pressures on parents of infants with these conditions. [ABSTRACT FROM AUTHOR]

Objective: To assess the impact of hypothermic neural rescue at birth on health-related quality of life (HRQL) in middle childhood.Design: Six-year to 7-year follow-up of surviving children from the Total Body Hypothermia for Neonatal Encephalopathy (TOBY) Trial.Setting: Community study including a single parental questionnaire to collect information on children's HRQL.Patients: 145 children (70 in the control group, 75 in the hypothermia group) whose parents consented and returned the questionnaire.Interventions: Intensive care with cooling of the body to 33.5°C for 72 hours or intensive care alone.Main Outcome Measures: HRQL attributes and utility scores using the Health Utilities Index (HUI).Results: At 6-7 years, speech appeared disproportionately affected when compared with other aspects of HRQL but levels of normal emotional functioning were similar in both groups. The mean (SE) HUI3 HRQL scores were 0.73 (0.05) in the hypothermia group and 0.62 (0.06) in the control group; mean difference (95% CI) 0.11 (-0.04 to 0.26).Conclusions: Findings of non-significant differences were not unexpected; the study used data from long-term survivors in a neonatal trial and was underpowered. However, results favoured moderate hypothermia and so complement the clinical results of the TOBY Children study. The work provides further insight into the long-term HRQL impact of perinatal asphyxial encephalopathy and provides previously unavailable utility data with which to contemplate the longer term cost-effectiveness of hypothermic neural rescue.Trial Registration Number: This study reports on the follow-up of the TOBY clinical trial: ClinicalTrials.gov number NCT01092637. [ABSTRACT FROM AUTHOR]

Objective: To evaluate England's NHS newborn sickle cell screening programme performance in children up to the age of 5 years.Design: Cohort of resident infants with sickle cell disease (SCD) born between 1 September 2010 and 31 August 2015 and followed until August 2016.Participants: 1317 infants with SCD were notified to the study from all centres in England and 1313 (99%) were followed up.Interventions: Early enrolment in clinical follow-up, parental education and routine penicillin prophylaxis.Main Outcome Measures: Age seen by a specialist clinician, age at prescription of penicillin prophylaxis and mortality.Results: All but two resident cases of SCD were identified through screening; one baby was enrolled in care after prenatal diagnosis; one baby whose parents refused newborn screening presented symptomatically. There were 1054/1313 (80.3%, 95% CI 78% to 82.4%) SCD cases seen by a specialist by 3 months of age and 1273/1313 (97%, 95% CI 95.9% to 97.8%) by 6 months. The percentage seen by 3 months increased from 77% in 2010 to 85.4% in 2015. 1038/1292 (80.3%, 95% CI 78.1% to 82.5%) were prescribed penicillin by 3 months of age and 1257/1292 (97.3%, 95% CI 96.3% to 98.1%) by 6 months. There were three SCD deaths <5 years caused by invasive pneumococcal disease (IPD) sensitive to penicillin.Conclusion: The SCD screening programme is effective at detecting affected infants. Enrolment into specialist care is timely but below the programme standards. Mortality is reducing but adherence to antibiotic prophylaxis remains important for IPD serotypes not in the current vaccine schedule. [ABSTRACT FROM AUTHOR]

Studies of adult patients have demonstrated that weekend admissions compared with weekday admissions had a significantly higher hospital mortality rate. We have reviewed the literature to determine if the timing of admission, for example, weekend or weekday, influenced mortality and morbidity in children. Seventeen studies reported the effect of timing of admission on mortality, and only four studies demonstrated an increase in those admitted at the weekend. Meta-analysis of the results of 15 of the studies demonstrated there was no significant weekend effect. There was, however, considerable heterogeneity in the studies. There were two large UK studies: one reported an increased mortality only for planned weekend admissions likely explained by planned admissions for complex conditions and the other showed no significant weekend effect. Two studies, one of which was large (n=2913), reported more surgical complications in infants undergoing weekend oesophageal atresia and trachea-oesophageal repair. Medication errors have also been reported to be more common at weekends. Five studies reported the effect of length of stay, meta-analysis demonstrated a significantly increased length of stay following a weekend admission, the mean difference was approximately 1 day. Those data, however, should be interpreted with the caveat that there was no adjustment in all of the studies for differences in disease severity. We conclude that weekend admission overall does not increase mortality but may be associated with a longer length of stay and, in certain conditions, with greater morbidity. [ABSTRACT FROM AUTHOR]