The disease course of children with ulcerative colitis (UC) varies substantially. Published data on predictors of disease outcomes in children remains scarce. We validate clinical predictors of outcomes in 93 children with UC in a single centre (age range: 2-18 years, minimum follow-up: 18 months). We stratified children into 3 groups according to their disease course, i.e. 1 = mild (38/93, 40.9%), 2 = moderate (38/93, 40.9%), 3 = severe (17, 18.2%). Comparison of clinical and biochemical parameters was performed between groups using Chi-square, Mann-Whitney and log-rank tests. Predictors of a severe disease course included pancolitis (P 0.01), low albumin (P 0.005), low haemoglobin at diagnosis (P 0.04), PUCAI at 3 months and non-response to steroids at 3 months (P 0.0001). In our cohort, failure to achieve remission at 3 months implied an 80% likelihood to require biologics or major surgery within 18 months. A specific 3 month review point is recommended to guide future management.

Biallelic variants in CACNA1A have previously been reported in nine individuals (four families) presenting with epilepsy and cognitive impairments of variable severity and age-of-onset. Here, we describe a child who presented at 6 months of age with drugresistant epilepsy and developmental delay. At 10 years of age, she has profound impairments in motor function and communication. MRI was initially unremarkable, but progressed to severe cerebellar atrophy by age 3 years. Next Generation Sequencing and panel analysis identified a maternally inherited truncating variant c.2042_2043delAG, p.(Gln681ArgfsTer100) and paternally inherited missense variant c.1693G>A, p.(Glu565Lys). In contrast to previously reported biallelic cases, parents carrying these monoallelic variants did not display clear signs of a CACNA1Aassociated syndrome. In conclusion, we provide further evidence that biallelic CAC-NA1A variants can cause a severe epileptic and developmental encephalopathy with progressive cerebellar atrophy, and highlight complexities of genetic counseling in such situations.

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Objectives Describe the changes in the patterns of paediatric respiratory admission observed in Hull University Teaching Hospitals (HUTH) during the first lockdown in the United Kingdom during the COVID-19 pandemic and explore the possible explanations for the observation. Methods The study included paediatric patients admitted to the HUTH with bronchiolitis, lower respiratory tract infections, asthma, viral-induced wheeze, or multi-trigger wheeze during the period of April 1st to May 31st in 2017, 2018, 2019, and 2020. Air pollution data was obtained as the Daily Air Quality Index. To study the well-being of patients who were under the care of the paediatric respiratory team, data on telephone respiratory clinics was also collected. General practitioner consultation data was sampled from six surgeries in Hull. Results Significant decline in paediatric respiratory admissions was observed in April and May of 2020 compared to the same months in previous the three years (decrease of 89.3% from 2017, 85.5% from 2018, and 87% from 2019). Data from the general practitioner surgeries revealed a decline in respiratory presentations. Findings from the telephone clinics revealed that most (87.2%) of the children under the paediatric respiratory team were doing well respiratory health perspective and did not require any changes to their treatment. Conclusions A significant decline in paediatric respiratory admissions was observed in HUTH during the first lockdown in the United Kingdom. Findings from telephone respiratory clinics and general practitioner consultations suggest that parental fear of contracting COVID-19 is unlikely to be the sole explanation for the observed decline.

methods if initial intubation is unsuccessful. Video laryngoscopy (VL) may allow faster time to best view and better views compared to direct laryngoscopy (DL) during intubation of the anatomically normal neonate. 2 Furthermore, VL can be effective for training purposes allowing real time feedback from senior colleagues and quicker acquisition of intubation skills. 3 Objectives The primary objective is to educate paediatricians to use VL. The secondary objective is to assess change in opinions and confidence in VL following simulation training. Methods Within a district general hospital, 17 participants including 12 paediatric trainees and 5 non-trainee participants (consultants, physicians associates and medical students). Each participant completed a pre and post simulation questionnaire, which included assessment of prior VL education and experience, understanding of planning for failure and confidence in VL technique. Low fidelity simulation training of VL (Mac-Grath) and airway adjuncts was undertaken by paediatric and anaesthetic colleagues. Results Of the participants, 8/17 (47%), including only 3/12 (25%) of the trainee group, had received previous training in VL, and 5/17 (29.4%) had previously used VL during real time intubation. In the pre-education group, 4/17 (23.5%) preferred initial intubation attempts using VL, which increased in post education group to 8/17 (47%). In the pre-education group, 6/17 (35.2%) stated they would choose VL for second intubation attempt, which increased in the post-education group to 15/17 (88.2%). In the pre-education group 4/18 (23.5%) stated they would be confident in using VL for second intubation attempt, which increased to 13/17 (76.4%) in the post-education group. Conclusions VL is a beneficial tool for neonatal intubation and non-invasive surfactant administration, and is a useful for allowing real-time feedback on the procedural skills from a supervising senior. In our study, we demonstrated that VL training and experience wasn't extensive, and following a short education programme, confidence and enthusiasm for VL increased.