Background: In congenital hyperinsulinism (CHI) there is dysregulation of insulin secretion that leads to hypoglycaemia. It is possible that other pancreatic hormones are also dysregulated in CHI.
Objectives: i) To verify the utility of Luminex Multiplex to determine pancreatic hormones in the paediatric age. ii) To investigate the response of pancreatic hormones (insulin, C-peptide, glucagon, amylin and PP) to a fast in children with CHI due to different underlying mechanisms.
Patients and methods: Plasma pancreatic hormones were collected in 12 children with CHI at a single centre in London (UK) at normoglycaemia and at hypoglycaemia (end of fast). The patients are five males and seven females with ages between 11 days of life and 13 years. Their CHI is due to different aetiologies, histology types and with different response to treatments. Hormones were analysed using multiplexing manner (Luminex Multiplex assay) on 0.025 ml of plasma.
Results: Insulin concentration (mean±SDS) decreases from 909±441 pg/ml in normoglucemia to 503±306 pg/ml at hypoglucemia (P 0.004). Similarly, C-peptide descends from 1547±1013 pg/ml to 806±366 pg/ml at normoglycaemia and hypoglycaemia, respectively (P 0.005). The concentrations of glucagon at these two time points are 97±239 and 103±260 pg/ml (P 0.21). Amylin decreases from 35±22 pg/ml to 21±9 pg/ml (P 0.014) whilst PP remains almost unchanged: 84±106 pg/ml at normoglycaemia and 86±115 pg/ml at hypoglycaemia (P 0.65).
Conclusions: This assay proves to be useful in determining pancreatic hormones in children. Glucagons response to hypoglycaemia is impaired in children with CHI. This is the first study to look at amylin concentrations in CHI. Amylin decreases during hypoglycaemia so as to avoid its anorectic effect, but interestingly PPs concentrations remain stable despite hypoglycaemia.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology