Integrin heterodimers containing an Integrin alpha V subunit are essential for development and play critical roles in cell adhesion and signaling. We identified biallelic variants in the gene coding for Integrin alpha V (ITGAV) in three independent families (two patients and four fetuses) that either caused abnormal mRNA and the loss of functional protein or caused mistargeting of the integrin. This led to eye and brain abnormalities, inflammatory bowel disease, immune dysregulation, and other developmental issues. Mechanistically, the reduction of functional Integrin αV resulted in the dysregulation of several pathways including TGF-β-dependent signaling and αVβ3-regulated immune signaling. These effects were confirmed using immunostaining, RNA sequencing, and functional studies in patient-derived cells. The genetic deletion of itgav in zebrafish recapitulated patient phenotypes including retinal and brain defects and the loss of microglia in early development as well as colitis in juvenile zebrafish with reduced SMAD3 expression and transcriptional regulation. Taken together, the ITGAV variants identified in this report caused a previously unknown human disease characterized by brain and developmental defects in the case of complete loss-of-function and atopy, neurodevelopmental defects, and colitis in cases of incomplete loss-of-function.

AimsDouble checking of medicine by two qualified nurses is implemented in paediatric practice to help prevent administration errors and is part of the medication policy for an Irish paediatric hospital.1 A four-day audit was completed in January 2019 to determine the compliance of the paediatric intensive care unit (PICU) staff with the double-checking policy. The results of this audit were presented at the weekly Multi-Disciplinary Team risk huddle. Awareness and education followed. The aim was to establish a baseline of documentation rates for the co-signing of administered medicines using an electronic healthcare record in a PICU and to compare rates before and after awareness sessions.MethodIn January 2019 for four non-consecutive days, the administration record for all patients on Floor one PICU were audited. This audit was repeated in July 2019. Using Philips® IntelliSpace Critical Care and Anaesthesia (ICCA), we counted the number of signatures for:Medication administration (all PO and IV medicines).Pump checks if required, according to the Standard Concentration Drug Library non-continuous table.Continuous infusion preparation and pump programming.Daily nurse handover checks.Compliance was calculated by dividing the number of documented checks divided by the number of checks required for each medicine. Using Microsoft Excel® graphs showing compliance rates were determined and a Chi-Squared Test undertaken.ResultsThere were 39 patients included and 520 medicine orders for January, and 43 patients and 595 medicine orders for July.There was an improvement of 8% in the co-signing for medicationPump checks improved by 20%Continuous infusions checks improved by 190%.Handover checks improved by 100%ConclusionThe results show a clear improvement of the compliance of PICU nurses to the co-signing method described by the CHI medication policy. This study has established a baseline rate against which future audits can be measured.Administration error detection rates can be improved with a double check.1 Anecdotally in PICU medications are being second checked, however, documentation on the electronic health record is poor, because of various barriers encountered by the nursing staff (lack of access to computers, time consuming log in). We found that by creating awareness at our weekly risk huddle, documentation of second checks improved.Double-checking has been shown to increase certain error detection rates in some circumstances, but not in others, especially if the age difference between the nurses is significant.2 Moreover, additional studies that consider the time cost of using the double-check method the impact on other patients of the unit, and the effect on the health professionals’ vigilance are needed. Because the double check is time-consuming and has limitations, it may best be reserved for only the highest-risk medications. It is also important to identify the barriers to double-checking that nurses encounter, such as computer availability, time spent logging on and off, etc. Those barriers should be explored with PICU staff.ReferencesAlsulami Z, Conroy S, Choonara I. A systematic review of the effectiveness of double checking in preventing medication errors. Arch Dis Child. 2012;97:e2.Douglass AM, Elder J, Watson R, et al. A randomized controlled trial on the effect of a double check on the detection of medication errors. Ann Emerg Med. 2018;71:74–82.e1.

AimsPaediatric delirium (PD) is a neuropsychiatric disorder with disrupted cerebral functioning due to underlying disease and/or as a result of critical care treatment.1 PD presents as hypoactive, hyperactive, or mixed, with hypoactive PD more commonly reported in children.2 PD is reported in 34% PICU admissions.3The European Society of Paediatric and Neonatal Critical Care Medicine (ESPNIC) and Society of Critical Care Medicine recommends screening in 100% of PICU patients from all age groups.3 4 As part of a quality improvement initiative (QI) PD screening was introduced to our hospital, with training completed on 11th March 2020. Ad hoc spot checks revealed screening rates less than 50%. An ethics waver by CHI research committee and permission from the PICU research group was granted in March 2022, to start a point prevalence once a week and feedback results to the PICU multidisciplinary risk meeting. MethodThe PICU is divided into two locations (floors). Patients on each floor were identified using the ward census book. Each electronic medical record (eHR) was accessed and screening compliance recorded using an MS Excel sheet.Data was collected retrospectively by a student on placement from the months March-October. ResultsOf the 565 patients records included, 146 (26%) had a morning PD score undertaken and 127 (22%) had an evening PD score undertaken on the day of audit. Compliance was higher in the morning for floor 1 (30%), with floor 2 having a higher compliance screening in the afternoon (27%). The highest compliance rates were recorded for both floors during May 2022. The highest compliance recorded was 46% for morning and 54% for afternoon screening.ConclusionsDespite formal education for all staff, and all new staff, PD screening rates remain low. Further work is required to identify strategies that could improve rates.ReferencesSchieveld JN, Leroy PL, van Os J, et al. Pediatric delirium in critical illness: phenomenology, clinical correlates and treatment response in 40 cases in the pediatric intensive care unit. Intensive Care Med 2007;33:1033–1040.Semple D, Howlett MM, Strawbridge JD, et al. A systematic review and pooled prevalence of delirium in critically ill children. Crit Care Med 2022;50:317–328.Harris J, Ramelet AS, van Dijk M, et al. Clinical recommendations for pain, sedation, withdrawal and delirium assessment in critically ill infants and children: an ESPNIC position statement for healthcare professionals. Intensive Care Med 2016;42:972–86. Smith HAB, Besunder JB, Betters KA, et al. 2022 Society of Critical Care Medicine Clinical Practice Guidelines on Prevention and Management of Pain, Agitation, Neuromuscular Blockade, and Delirium in Critically Ill Pediatric Patients With Consideration of the ICU Environment and Early Mobility. Pediatr Crit Care Med 2022;23:e74–e110.

Contemporary therapeutic approaches to trauma include an understanding that traumatic experiences are stored implicitly in the body and that the body may ‘narrate’ these stories through physical symptoms or bodily enactments. In this article, the author reflects on her experience as an art therapist at a specialist therapeutic unit, where she works with young people and families affected by sexual abuse and sexual violence. It weaves together reflections on various personal encounters with the performing and visual arts (outside of the therapy space) that have offered a different lens on themes that regularly surface in clinical work. A selection of images drawn from these engagements with the arts are discussed here, with reference to the endeavour in therapy to support the traumatized body towards healing and recovery.

Abstract Methotrexate is a vital medication in treating different types of malignancies. There are few reports of successful administration of intravenous methotrexate post adverse reaction by step-up desensitization. We share here, our experience in overcoming methotrexate hypersensitivity though administration of methotrexate by fixed rate infusion in an allergic child who failed a step-up methotrexate desensitization protocol. The outcome of this case suggests an underlying, non IgE mediated mechanism for the adverse reaction.

BackgroundNitrous oxide (N2O) has multiple benefits in paediatric procedural sedation (PPS), but use is restricted by its limited analgesic properties. Analgesic potency could be increased by combining N2O and intranasal fentanyl (INF). We assessed safety and efficacy data from 10 years (2011–2021) of our N2O PPS programme.MethodsProspectively collected data from a sedation registry at a paediatric emergency department (PED) were reviewed. Total procedures performed with N2O alone or with INF, success rate, sedation depth and adverse events were determined. Contributing factors for these outcomes were assessed via regression analysis and compared between different N2O concentrations, N2O in combination with INF, and for physician versus nurse administered sedation. A post hoc analysis on factors associated with vomiting was also performed.Results831 N2O procedural sedations were performed, 358 (43.1%) involved a combination INF and N2O. Nurses managed sedation in 728 (87.6%) cases. Median sedation depth on the University of Michigan Sedation Scale was 1 (IQR 1–2). Sedation was successful in 809 (97.4%) cases. Combination INF/N2O demonstrated higher median sedation scores (2 vs 1, p<0.001) and increased vomiting (RR 1.8, 95% CI 1.3 to 2.5), with no difference in sedation success compared with N2O alone. No serious adverse events (SAEs) were reported (desaturation, apnoea, aspiration, bradycardia or hypotension) regardless of N2O concentration or use of INF. 137 (16.5%) minor adverse events occurred. Vomiting occurred in 113 (13.6%) cases and was associated with higher concentrations of N2O and INF use, but not associated with fasting status. There were no differences in adverse events (RR 0.98, 95% CI 0.97 to 1.04) or success rates (RR 0.93, 95% CI 0.56 to 1.7) between physician provided and nurse provided sedation.ConclusionN2O can provide effective PED PPS. No SAEs were recorded. INF may be an effective PPS adjunct but remains limited by increased rates of vomiting.

ObjectiveTo provide recommendations for a comprehensive management approach for infants and children presenting with symptoms or signs of aspiration. MethodsThree rounds of surveys were sent to authors from 23 institutions worldwide. The threshold for the critical level of agreement among respondents was set at 80 %. To develop the definition of “intractable aspiration,” each author was first asked to define the condition. Second, each author was asked to complete a 5-point Likert scale to specify the level of agreement with the definition derived in the first step. ResultsRecommendations by the authors regarding the clinical presentation, diagnostic considerations, and medical and surgical management options for aspiration in children. ConclusionApproach to pediatric aspiration is best achieved by implementing a multidisciplinary approach with a comprehensive investigation strategy and different treatment options.

Type 1 diabetes-management can be considered an adolescent-parent collaboration. Given particular adolescent adherence challenges, it is integral that adolescent-parent dyadic relationships are investigated. Therefore, this study aimed to explore dyads' adjustment to type 1 diabetes, while examining the congruence/dissimilarity within these dyads. Semi-structured interviews were conducted with 10 dyads (20 individuals) separately. Interviews were transcribed verbatim and analysed with thematic analysis using a dyadic framework method. Findings suggested complex experiences of adjustment among parents and adolescents which reflect two main themes - Never-Ending Abyss of Management and Diabetes Integration, with three subthemes - A Life of Food Restrictions, Evolving Familial Bonds and Technology as easing the burden of Diabetes. Dyadic analyses revealed dyadic congruence across most themes. This study adds to the adjustment literature by providing a systemic perspective rarely presented in prior paediatric research.