Objectives Acute‐on‐chronic liver failure (ACLF) is an acute decompensation of cirrhosis complicated by other organ failure and is associated with increased mortality and morbidity. ACLF has not been studied in children with biliary atresia (BA), which is the commonest indication for pediatric liver transplantation (LT) worldwide. This study aims to evaluate ACLF and outcomes in children with BA while awaiting deceased donor LT. Methods This was a subanalysis of the dataset from a prospective cohort study of patients aged 0‐18 years who underwent portoenterostomy for BA and were listed for LT at King's College Hospital, London, between 1999 and 2003. Outcomes included the development of ACLF, mortality, and complications. Results Ninety‐nine (41 male) children were included, and follow‐up was 10 [6.0‐15.0] years. A total of 20/99 children developed ACLF. ACLF was associated with increased mortality while awaiting LT (20% vs 4%; P = 0.03). There were no associations between biochemical parameters at listing and death. Increased bilirubin levels 3 months post‐portoenterostomy was predictive of development of ACLF (AUROC = 0.72, P < 0.01). Age at LT and time on the waiting list in the ACLF subgroup were both lower compared to the non‐ACLF group (P > 0.05). Sepsis and gastrointestinal bleeding were the commonest precipitants of ACLF. Complications included ascites, hepatic encephalopathy, and hepatorenal syndrome; the ACLF subgroup required multisystem support and longer intensive care unit stay. Conclusions ACLF in children with BA awaiting deceased donor LT carries increased mortality and morbidity. This warrants stratification of patients for earlier wait‐listing and prioritization for LT.

Long-term graft fibrosis occurs in the majority of pediatric liver transplant recipients. Serial biopsies to monitor graft health are impractical and invasive. The APRI has been evaluated in pediatric liver disease, but not in the context of post-transplantation fibrosis. We aimed to investigate the validity of APRI as a predictor of long-term graft fibrosis in pediatric liver transplant recipients. This was a retrospective, observational study of a cohort of children who underwent liver transplantation at King's College Hospital between 1989 and 2003, with a relevant dataset available. Protocol liver biopsies were performed at 10-yr follow-up and fibrosis was graded using the Ishak scoring system, with S3-6 denoting "significant fibrosis." APRI was calculated concurrently with biopsy. A total of 39 asymptomatic patients (20 males; median age at transplant, 1.43 yr) underwent protocol liver biopsies at a median of 10.39 yr post-transplantation. APRI was associated with significant fibrosis (p = 0.012). AUROC for APRI as a predictor of significant fibrosis was 0.74 (p = 0.013). The optimal cutoff APRI value for significant fibrosis was 0.45 (sensitivity = 0.67; specificity = 0.79; PPV = 0.67; NPV = 0.79). APRI appears to be a useful non-invasive adjunct in the assessment of significant graft fibrosis in the long-term follow-up of pediatric liver transplant survivors.

ObjectivesTo evaluate the usefulness and effectiveness of a new structured education module for children with type 1 diabetes: Structured Education Reassuring Empowering Nurturing (SEREN) ‘Diabetes at Diagnosis’.DesignRetrospective questionnaire-based service evaluation.Setting12/14 paediatric diabetes centres across Wales took part.ParticipantsChildren diagnosed with type 1 diabetes 1 year before (pre-SEREN group) and 1 year after the introduction of SEREN (post-SEREN group) were selected using a national diabetes register.Resource‘Diabetes at Diagnosis’ delivers structured education to empower children and families with self-management of type 1 diabetes.EvaluationPrimary outcomes were patient-reported effectiveness and user-friendliness of the educational resources and quality of life (PedsQL). Age-appropriate child and parent questionnaires were provided. Clinical outcomes included glycated haemoglobin (HbA1c) at 6 and 12 months, service engagement and diabetes-related hospital admissions in the first year.Results89/106 responded pre-SEREN and 108/115 post-SEREN, with no demographic differences at diagnosis. Parent scores for educational package evaluation significantly improved post-SEREN, with a non-significant trend towards improved results in children. PedsQL scores were similar. There was no change in HbA1c overall. Subgroup analyses at 12 months showed a trend towards a lower HbA1c in key stage 1–2 (62 vs 58 mmol/mol, p=0.06) and increased HbA1c in key stage 3–4 (56 vs 66 mmol/mol, p=0.009). There were no differences in hospital admissions or missed clinic appointments.ConclusionsThis is an evaluation of the only standardised type 1 diabetes structured education programme in use for children throughout Wales. This module improved parent-reported outcomes and showed a non-significant trend towards improved usefulness in children, without a difference in a PedsQL scores overall. Ongoing evaluation of the cohort who received subsequent SEREN modules may show the long-term benefit of the programme.

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