Insulinoma in childhood: a retrospective review of 22 patients from one referral centre.

Background: Insulinomas are very rare in childhood with sparse knowledge on the clinical aspects and the presence of Multiple Endocrine Neoplasia type 1 (MEN1). Methods: We conducted a retrospective review of patients diagnosed with insulinoma between 1995 and 2021, presenting to one referral centre in Russia. Clinical, biochemical, genetic, imaging and histological data were collected. In addition, follow-up and family data were obtained. Results: A total of twenty-two children aged 5 to 16 years were identified. The median (range) gap between the first hypoglycaemia symptoms and diagnosis was 10 (1-46) months. Twelve children (55%) were misdiagnosed to have epilepsy and were treated with anticonvulsants before hypoglycemia was revealed. Contrast enhanced MRI and/or CT were accurate to localize the lesion in 82% (n=18). Five patients (23%) had multiple pancreatic lesions. All children underwent surgical treatment. The median (range) diameter of removed tumors was 1.5 (0.3-6) cm. Histopathological studies confirmed the presence of insulinoma in all cases. Immunohistochemical studies revealed G2 differentiation grade in 10 out of 17 cases. Two patients were diagnosed with metastatic insulinoma. One of them had metastases at the time of insulinoma diagnosis, while the other was diagnosed with liver metastases eight years after the surgery. Eight children (36%) were found to carry MEN1 mutations, inherited n=5, de novo n=1, no data, n=2. Children with MEN1 had significantly higher number of pancreatic tumors compared to sporadic cases. All of them developed additional MEN1 symptoms during the following 2-13 years. In the five patients with inherited MEN1, seven family members had hitherto undiscovered MEN1 manifestations. Conclusions: In this large cohort of children with rare pediatric insulinomas, MEN1 syndrome and G2 tumors were frequent, as well as hitherto undiscovered MEN1 manifestations in family members. Our data emphasize the need of genetic testing in all children with insulinoma and their relatives, even in the absence of any other features, as well as the importance of a prolonged follow-up observation.

The Managing Emergencies in Paediatric Anaesthesia global rating scale is a reliable tool for simulation-based assessment in pediatric anesthesia crisis management.

INTRODUCTION: The use of simulation-based assessments for high-stakes physician examinations remains controversial. The Managing Emergencies in Paediatric Anaesthesia course uses simulation to teach evidence-based management of anesthesia crises to trainee anesthetists in the United Kingdom (UK) and Canada. In this study, we investigated the feasibility and reliability of custom-designed scenario-specific performance checklists and a global rating scale (GRS) assessing readiness for independent practice. METHODS: After research ethics board approval, subjects were videoed managing simulated pediatric anesthesia crises in a single Canadian teaching hospital. Each subject was randomized to two of six different scenarios. All 60 scenarios were subsequently rated by four blinded raters (two in the UK, two in Canada) using the checklists and GRS. The actual and predicted reliability of the tools was calculated for different numbers of raters using the intraclass correlation coefficient (ICC) and the Spearman-Brown prophecy formula. RESULTS: Average measures ICCs ranged from 'substantial' to 'near perfect' (P ≤ 0.001). The reliability of the checklists and the GRS was similar. Single measures ICCs showed more variability than average measures ICC. At least two raters would be required to achieve acceptable reliability. CONCLUSIONS: We have established the reliability of a GRS to assess the management of simulated crisis scenarios in pediatric anesthesia, and this tool is feasible within the setting of a research study. The global rating scale allows raters to make a judgement regarding a participant's readiness for independent practice. These tools may be used in the future research examining simulation-based assessment.