PandAcuity in paediatrics: a novel clinical measure of visual function based on the panda illusion

Background/aimsTo evaluate the PandAcuity test for visual function testing in a paediatric cohort and to examine its agreement with conventional visual acuity (VA) testing.MethodsPandAcuity scores were determined in 152 children (77 males) aged between 3 and 15 years after VA testing (LEATM-test, E-chart, Landolt-C-rings or numbers). The PandAcuity test consisted of illusions made up from silhouettes of animals ‘hidden’ within zig-zag-patterns of decreasing spatial frequencies. Correlation analyses between PandAcuity score and VA were performed.Results150 children completed the test in at least one eye, 148 in both eyes. The PandAcuity test demonstrated good test–retest reliability (intraclass correlation coefficient=0.89) between two runs. VA and PandAcuity score showed a medium to large correlation (Spearman’s ρ=0.52, p<0.0001). 93% of the children’s visual impairment was classified in the same range by both test types. Receiver operating characteristic analysis of predicted visual impairment showed an excellent agreement with the classification based on VA testing (AUC=0.84).ConclusionThe PandAcuity test is rapid, simple and well accepted, rendering it a suitable supplement for the clinical assessment of VA in children. Because of its counterintuitive application (a higher number of correctly identified images means worse VA), it can be used to cross-validate conventional acuity tests to assure children’s compliance.

Chronic nonbacterial osteomyelitis in children: a multicentre Belgian cohort of 30 children

BackgroundTo evaluate clinical characteristics, imaging findings, therapeutic approach and outcome of paediatric patients with Chronic Non-Bacterial Osteomyelitis (CNO). MethodsRetrospective review of 30 children diagnosed with CNO at two tertiary care centres in Belgium. Imaging data were evaluated by blinded paediatric radiologists. ResultsMean age at onset was 10.3 years and mean age at diagnosis was 11.7 years. Bone pain was the leading symptom (29/30 patients). Out of 180 symptomatic lesions, 131 were confirmed on MRI as hyperintense geographic lesions on STIR images at the metaphysis and epiphysis adjacent to growth plates of tubular bones. The most common sites of involvement were the lower limbs, spine, sternoclavicular joint and humerus. For nearly half of the patients (14/30) monotherapy with NSAIDs was sufficient to obtain remission. The remaining 16 patients received second-line therapy: bisphosphonates (n=15/30), disease-modifying antirheumatic drugs (n=7/30), etanercept (n=4/30) and tocilizumab (n=1/30). 26/30 Patients reached remission after a mean time of 37.6 months. The prognosis was worse for patients with spinal involvement, resulting in more long-term sequelae. ConclusionsWe present a multicentre paediatric cohort of 30 CNO patients. A typical pattern of bone involvement could be found on MRI. NSAIDs were administered as first-line treatment. Second-line strategies included bisphosphonates, corticosteroids, methotrexate, etanercept and tocilizumab. Trial registrationRetrospectively registered.

Pulmonary arterial hypertension associated with congenital heart disease in children: clinical characterisation, outcomes and changes in demographics over time

Background/Introduction Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) in children and is associated with significant morbidity and mortality. The impact of different phenotypes on management and survival remains unclear. Purpose To examine the clinical features, management and outcomes of paediatric PAH-CHD patients based on the 20-year experience of the UK National Paediatric Pulmonary Hypertension (PH) Service. Methods Consecutive PAH-CHD patients entering the service between January 2001 and January 2021 were included and classified into: Eisenmenger syndrome (ES, group A), PAH related to a significant systemic-pulmonary shunt (group B), PAH with small or co-incidental CHD (group C) and PAH following defect repair (group D). Incident patients (without pre-existing PAH) were included in survival analysis. Results Of the overall PH paediatric cohort of 1104 patients, 819 (74.2%) had co-existing CHD and 354 (32.1%) patients received a diagnosis of PAH-CHD: 57.1% female, median [IQR] 4.6 [1.7–10.9] years. Group D PAH-CHD was the commonest subgroup, accounting for 36%, while the least frequent subtype was group C (14%). Group A and group B PAH-CHD represented 26% and 24%, respectively. Down syndrome was present in over one third (122, 34.5%) of PAH-CHD patients and was more commonly associated with ES (p=0.02). PAH therapy was started in 79.9% of PAH-CHD patients. At the end of follow-up, patients with group C PAH-CHD were more likely to be on combination therapy than any other group (64.6% vs. 28.4%, p&lt;0.0001). Prostanoid therapy was used in a minority (11%) of patients. The subgroup distribution of PAH-CHD at diagnosis changed from the early (2000–2005) to late (2015–2020) period (Figure 1). The proportion of ES patients decreased from 43.4% to 14.6% of PAH-CHD (p&lt;0.0001). The proportion of group B PAH-CHD patients increased (9.4% vs. 33.3%, p&lt;0.0001), with the majority (59.3%) deemed “operable” on specialist assessment. There was a trend for an increase in repaired PAH-CHD patients between the early and late era (31.1% vs. 43.8%, p=0.09). Transplant-free survival in PAH-CHD was 90.9% (95% CI: 87.8–94%) at 1 year, 77.9% (95% CI: 73.1–83.1%) at 5 years, and 74.9% (95% CI: 69.6–80.7%) at 10 years (Figure 2). Group C PAH-CHD had a lower transplant-free survival than the other 3 groups (HR 2.54, 95% CI: 1.51–4.28, p=0.0005). There was no difference in outcome between group A and group D PAH-CHD (HR 1.19, 95% CI: 0.62–2.28, p=0.6). Conclusions Repaired PAH-CHD, not ES, was the most common subtype in this large paediatric cohort. Over time, there were fewer ES patients and more “operable” patients with left-right shunts, suggesting an improvement in early diagnosis and management. Despite widespread use of PAH therapy, PAH-CHD remains a life-limiting disease with the poorest outcomes in PAH with co-incidental CHD. FUNDunding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Dr Constantine received a personal PhD fellowship grant (CHAMPION PhD Fellowship) Figure 1 Figure 2

178 Outcomes After Paediatric Extracorporeal Cardiopulmonary Resuscitation (ECPR) In A Tertiary Centre

Objective To report our outcomes in utilizing extracorporeal membrane oxygenation (ECMO) institution for cardiac arrest. Method Retrospective records of all patients that needed ECPR between January 2015 and July 2020 have been reviewed. Primary outcomes were survival to ECMO decannulation, hospital discharge and one year survival. Secondary outcomes were the need for ECMO re-cannulation and neurology outcome using Paediatric Cerebral Performance Category (PCPC). Results A total of 44 consecutive patients were identified. 75 % were post cardiac surgery (n = 33), mean time from arrest to initiation of ECMO was 39.5 mins +/- 17.7 mins. Mean highest lactate prior to ECMO was 12.9 +/- 4.4. 79.6 % of patients received central cannulation. 11.4 % of patients (n = 5) needed more than one run of ECMO. Mean hours on ECMO were 175.4 +/- 212.5 hours and mean PCPC score was 2.14 +/- 1.68. Mean ICU stay was 16.2 +/- 16.9 days and total hospital stay was 47 +/- 68.5 days. Overall Survival to ECMO weaning was 68.4 % (n = 13) vs 92 % (n = 23) in neonates and paediatric patients, respectively. Survival to hospital discharge was 47.4 % (n = 9) vs 72 % (n = 18) and one year survival was 42.1 % (n = 8) vs 72 % (n = 18) in the neonatal and paediatric cohort, respectively. Conclusions Our survival rates are encouraging and in line with current published literature and comparable favourably to International ELSO (Extracorporeal life support organisation) registry for neonates and paediatric patients of all cause ECPR. Paediatric patients showed a survival advantage over neonates after ECPR.

Effects of repeated vaccination and vaccine formulation on the induction of broadly neutralizing antibody responses against influenza A virus in children

The induction of broadly neutralizing antibodies (bNAbs) that target the hemagglutinin stalk domain is a promising strategy for the development of universal influenza virus vaccines. bNAbs can be boosted in adults by sequential exposure to heterosubtypic viruses through natural infection or vaccination. However, little is known about if/how bNAbs are induced by vaccination in more immunologically naive children. Here, we describe the impact of repeated seasonal influenza vaccination and vaccine type on induction of bNAbs in a well-characterized paediatric cohort enrolled in a series of randomized control trials of seasonal influenza vaccination. Repeated seasonal vaccination resulted in significant boosting of a durable bNAb response. Boosting of serological bNAbs titers was comparable within inactivated and live attenuated (LAIV) vaccinees and declined with age. These data provide new insights into vaccine-elicited bNAb induction in children, which has important implications for the design of universal influenza vaccine modalities in this critical population.