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Objectives The primary objective was to define the incidence of JSLE in children <16 years of age in the UK and Republic of Ireland (ROI). The secondary objective was to describe presenting features, classification criteria, initial management and disease damage in newly presenting JSLE patients. Methods A prospective JSLE epidemiological study was undertaken between September 2017 and September 2019 with support of the British Paediatric Surveillance Unit and other professional groups involved in diagnosis and management of JSLE patients. Treating consultants reported all cases of JSLE seen. A follow-up study at 1 year examined management and progression of disease and treatment. Results There were 124 incident cases included in the final analysis. Incidence was estimated using ACR-1997 classification criteria (0.36/100 000), SLICC-2012 classification criteria (0.41/100 000) and clinician expert opinion (0.46/100 000). A high disease burden was seen, with 71.0% of patients requiring ongoing systemic CS treatment at 1 year; 98.2% receiving immunomodulatory treatment; and 20.4% accruing damage in the year following diagnosis (predominantly neuropsychiatric-related), with substantial involvement from multiple speciality teams. Conclusions The minimum UK and ROI incidence of JSLE is between 0.36 and 0.46/100 000, depending on the case definition used. Challenges in classification of patients with JSLE are highlighted, but overall this study supports the use of SLICC-2012 classification criteria. The high levels of disease damage and ongoing CS use 1 year after diagnosis is concerning, highlighting the need for further interventions to improve outcomes in JSLE. [ABSTRACT FROM AUTHOR]

Background: Point-of-care testing (POCT) is diagnostic testing performed at or near to the site of the patient. Understanding the current capacity, and scope, of POCT in this setting is essential in order to respond to new research evidence which may lead to wide implementation.Methods: A cross-sectional online survey study of POCT use was conducted between 6th January and 2nd February 2020 on behalf of two United Kingdom (UK) and Ireland-based paediatric research networks (Paediatric Emergency Research UK and Ireland, and General and Adolescent Paediatric Research UK and Ireland).Results: In total 91/109 (83.5%) sites responded, with some respondents providing details for multiple units on their site based on network membership (139 units in total). The most commonly performed POCT were blood sugar (137/139; 98.6%), urinalysis (134/139; 96.4%) and blood gas analysis (132/139; 95%). The use of POCT for Influenza/Respiratory Syncytial Virus (RSV) (45/139; 32.4%, 41/139; 29.5%), C-Reactive Protein (CRP) (13/139; 9.4%), Procalcitonin (PCT) (2/139; 1.4%) and Group A Streptococcus (5/139; 3.6%) and was relatively low. Obstacles to the introduction of new POCT included resources and infrastructure to support test performance and quality assurance.Conclusion: This survey demonstrates significant consensus in POCT practice in the UK and Ireland but highlights specific inequity in newer biomarkers, some which do not have support from national guidance. A clear strategy to overcome the key obstacles of funding, evidence base, and standardising variation will be essential if there is a drive toward increasing implementation of POCT. [ABSTRACT FROM AUTHOR]

Acetylsalicylic acid is a frequently used medication worldwide. It is not used in pediatrics due its association with Reye syndrome. However, in case of pediatric intoxication, children are more fragile to salicylate poisoning because of their reduced ability of buffer the acid stress. Intoxication leads to a decoupling of oxidative phosphorylation and subsequently to a loss in mitochondrial function. Symptoms of poisoning are diverse; eventually they can lead to the death of the patient. Governmental websites of various EU countries were searched for legal information on acetylsalicylic acid availability in pharmacies and non-pharmacy stores. Various EU countries permit prescription-free sales of acetylsalicylic acid in pharmacies and non-pharmacy stores. In Sweden acetylsalicylic acid 500 mg may be sold in a maximum package size of 20 tablets or effervescent tablets in a non-pharmacy. In the UK a maximum of 16 tablets of acetylsalicylic acid 325 mg is allowed to sell in non-pharmacies. In Ireland acetylsalicylic acid is classified as S2 medication. Subsequently, acetylsalicylic acid is allowed to be sold prescription-free in pharmacies and non-pharmacy stores. In the Netherlands acetylsalicylic acid may only be sold in drug stores or pharmacies. A maximum of 24 tablets of 500 mg is allowed to purchase in a drug store. Several countries in the European Union are permitted to offer acetylsalicylic acid prescription-free in pharmacies and non-pharmacy stores without legal guidance on the storage position within the store. Further research is needed to investigate whether acetylsalicylic acid is located directly accessible to young children within the stores in EU countries which permit prescription-free sales of acetylsalicylic acid. [ABSTRACT FROM AUTHOR]

Paracetamol is used widely in pediatrics because it has a high drug safety when used in therapeutic dosages. In case of overdose the majority of paracetamol is metabolized to N-acetyl-p-benzoquinone imine (NAPQI), which is responsible for the severe toxic effects. The covalent connection between NAPQI and hepatic proteins leads to hepatocellular damage and possibly to severe liver failure. The antidote for paracetamol is N-acetylcysteine (NAC). It is a precursor of glutathione and aids to fill glutathione stores. The Rumack-Matthew nomogram should be used to decide on antidote treatment. Pediatric drug metabolism differs from adult metabolism. Children have a larger liver size compared to their body weight than adults, resulting in a higher metabolism rate. Young children seem to be less sensitive to acute intoxication than adults. One hypothesis to explain the lower rate refers to the larger liver size. The acute toxic dosage for children is more than 200 mg/kg body weight. There seems to be a global increase in accidental pediatric paracetamol overdose. Governmental websites of various European Union (EU) countries were searched for legal information on paracetamol availability in pharmacies and non-pharmacy stores. Various EU countries permit prescription-free sales of paracetamol in pharmacies and non-pharmacy stores. In Sweden paracetamol 500 mg may be sold in both pharmacies and non-pharmacies in a maximum pack size of 20 units. In the United Kingdom (UK) paracetamol 500 mg is listed in the general sales list with a maximum pack size of 30 effervescent tablets or 16 tablets. In Ireland paracetamol 500 mg may be sold in a maximum pack size of 12 units in a non-pharmacy. In the Netherlands paracetamol 500 mg is legal to be sold in a maximum pack size of 50 units in a drug store and with a maximum of 20 units in any other non-pharmacy. Several countries in the European Union are permitted to offer paracetamol prescription-free in pharmacies and non-pharmacy stores without legal guidance on the storage position within the store. Further research is needed to investigate whether paracetamol is located directly accessible to young children within the stores in EU countries which permit prescription-free sales of paracetamol. [ABSTRACT FROM AUTHOR]

The aim of the present study was to describe the present knowledge of healthcare professionals and the practices surrounding enteral feeding in the UK and Irish paediatric intensive care unit (PICU) and propose recommendations for practice and research. A cross-sectional (thirty-four item) survey was sent to all PICU listed in the Paediatric Intensive Care Audit Network (PICANET) database (http://www.picanet.org.uk) in November 2010. The overall PICU response rate was 90 % (27/30 PICU; 108 individual responses in total). The overall breakdown of the professional groups was 59 % nursing staff (most were children's nurses), 27 % medical staff, 13 % dietitians and 1 % physician assistants. Most units (96 %) had some written guidance (although brief and generic) on enteral nutrition (EN); 85 % of staff, across all professional groups (P= 0·672), thought that guidelines helped to improve energy delivery in the PICU. Factors contributing to reduced energy delivery included: fluid-restrictive policies (60 %), the child just being ‘too ill’ to feed (17 %), surgical post-operative orders (16 %), nursing staff being too slow in starting feeds (7 %), frequent procedures requiring fasting (7 %) and haemodynamic instability (7 %). What constituted an ‘acceptable’ level of gastric residual volume (GRV) varied markedly across respondents, but GRV featured prominently in the decision to both stop EN and to determine feed tolerance and was similar for all professional groups. There was considerable variation across respondents about which procedures required fasting and the duration of this fasting. The present survey has highlighted the variability of the present enteral feeding practices across the UK and Ireland, particularly with regard to the use of GRV and fasting for procedures. The present study highlights a number of recommendations for both practice and research. [ABSTRACT FROM PUBLISHER]

Objectives To investigate whether prenatal screening is effective in the detection of total anomalous pulmonary venous connection (TAPVC) and to identify common prenatal features. Methods This was a retrospective collaborative study involving 19 pediatric cardiac centers in the UK, Ireland and Sweden. Cases with TAPVC born between January 1, 1998 and December 31, 2004, and prenatally diagnosed cases whose estimated dates of delivery were within this time frame, were identified. Cases with functionally univentricular circulation or atrial isomerism were excluded. All available data and stored images were reviewed. Results Four-hundred and twenty-four cases with TAPVC were identified prenatally or postnatally, of whom eight (1.9%) had a prenatal diagnosis of TAPVC. Median gestational age at fetal diagnosis was 26 + 6 (range, 22 + 4 to 32 + 0) weeks. Six further fetuses with TAPVC had an abnormality diagnosed on prenatal ultrasound, but not the TAPVC. This included other congenital heart defects (four cases) and isolated pleural effusion (two cases). Seventeen (4.0%) of the 422 liveborn infants had a first-degree relative with congenital heart disease; and six of 17 had a sibling with TAPVC. Two died in utero. Of the liveborn infants diagnosed prenatally with TAPVC, none required urgent intervention for pulmonary venous obstruction and all were alive and well at a median of 2.3 (range, 1.0-7.0) years after surgical repair. Conclusion Prenatal diagnosis of TAPVC is infrequent using current screening methods. Where there is a family history of TAPVC, specialized fetal echocardiography at 20 and 28 weeks' gestation may be indicated. Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Objective: To conduct and report monitoring of vitamin K deficiency bleeding (VKDB) in Great Britain and Ireland following the 1988–90 survey (VKDB-90). Design: Two 2-year surveys conducted during 1993–4 (VKDB-94) and 2001–02 (VKDB-02). Setting: Data collected from all consultant paediatricians in Great Britain and Ireland. Patients: All infants presenting with bleeding resulting from vitamin K (VK) deficiency. Main outcome measures: Incidence of VKDB, related mortality/morbidity and VK prophylaxis recommended/received, noting predisposing features. Results: Compared with previous studies, VKDB-02 found fewer cases of VKDB (RR: 0.27 (95% CI: 0.12 to 0.59), p<0.001) with no deaths, no long-term morbidity and reduced incidence among those receiving any oral dosing (RR: 0.24 (95% CI: 0.06 to 1 .01), p<0.059). Breast-fed infants accounted for the vast majority of cases. The number receiving no prophylaxis fell consecutively over time: 20 of 27 in VKDB-90, 10 of 32 in YKDB-94 and 4 (because of parental refusal) of 7 in VKDB-02. Seven received one oral dose of VK in VKDB- 90, 16 in YKDB-94 and none in VKDB-02. Underlying liver disease was found in six cases in VKDB-90, 12 in VKDB-94 and one in VKDB-02. Conclusions: In the most recent survey, the incidence of VKDB was about one third that in the two earlier studies. Late onset VKDB remains virtually confined to breast-fed infants who have received either no VK or just one oral dose. The effectiveness of oral prophylaxis regimens has improved over the last 15 years, but parental refusal of prophylaxis has become more problematic. [ABSTRACT FROM AUTHOR]

Poster presentation Tuesday 8 October Background Sjögren's syndrome, a chronic multisystem autoimmune disease is characterised by inflammation of the exocrine glands, principally the salivary and lacrimal glands resulting in xerostomia and xerophthalmia. It can also present with more extensive exocrinopathy as well as extra-glandular, systemic features. Defined as primary SS (pSS) when there is no association with other autoimmune disease, reported incidence and prevalence rates vary. Juvenile-onset pSS is believed to be rare; however it is likely that it is under-recognised and therefore under-diagnosed. To date there have been no studies reporting accurate incidence or prevalence of paediatric pSS (PpSS). Diagnosing pSS can be challenging. Many of the cardinal symptoms are non-specific and no gold standard biomarker of disease exists. Between 1965–2002 eleven diagnostic criteria sets were developed, none of which have gained universal acceptance or been validated for use in a paediatric population. Until recently, the most widely used criteria were those developed by the American-European Consensus Group. It remains well-recognised that international consensus on classification is important for standardisation, particularly in relation to research and monitoring treatment outcomes. With this in mind, the 2016 ACR/EULAR criteria were developed. However, there still remains a paucity of validated classification criteria for diagnosis of juvenile-onset pSS. Paediatric-focused criteria are required as features of pSS in children differ from those observed in adults. Children experience less dryness and more frequently experience systemic symptoms and parotid enlargement. Hence, simply applying adult criteria to a paediatric population may lead to mis- and/or under-diagnosis. The overarching aim of this study is to identify epidemiological, clinical and laboratory characteristics of PpSS in a multi-centre cohort of patients. Using this data our goal is to develop universally accepted classification criteria validated for use in a paediatric population. Going forward, this would enable standardisation of PpSS classification allowing for robust studies into disease pathogenesis, management and prognosis. Methods Inclusion criterion for entry into the UK/Ireland PpSS cohort study and repository is diagnosis of pSS before 18 years. A data collection pack will be sent to authors willing to participate. Information collected will include demographic, clinical and laboratory/histological data at diagnosis and subsequent follow-up appointments. Biological samples including blood, tears, saliva, urine, and glandular and extra-glandular (e.g. renal) tissue will be collected prospectively if available. Outcome measures related to disease activity and damage, as well as patient reported outcomes will also be collected at set time-points. Results No results to report. Conclusion The UK/Ireland PpSS cohort study and repository will capture data on a significant cohort of children with pSS providing a powerful resource to help improve our understanding of this rare disease. Prospective data collection will allow a fuller analysis of poor prognostic features, impact of therapy, damage accrual and variable outcome of PpSS. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]