Background: Hyperinsulinism is a common cause of persistent hypoglycaemia in infant. Insulin secretion from pancreatic β-cells is unregulated and inappropriate for the level of blood glucose, causing glucose into the insulin-sensitive tissues, such as the muscle, liver and adipose tissue, leading to hyperinsulinaemic hypoglycaemia. But congenital portosystemic shunt, a rare vascular malformation, can cause hyperinsulinaemic hypoglycaemia rarely because glucose from portal to systemic circulation causes early hyperglycaemia leading to exaggerated insulin secretion, leading to bypassing the hepatic metabolism directly entering into the systemic circulation, which results in hyperinsulinaemia, then in turn causes late hypoglycaemia.
Case: We report a rare case of hyperinsulinaemic hypoglycaemia secondary to a congenital portosystemic shunt in a newborn. A 1-day-old female was referred our hospital for hypoglycaemia without response in i.v. 10% glucose infusion. She was born at 38 weeks via caesarean section and second child of healthy parents. Her birth weight was 3.3 kg. The patient developed dyspnea after birth, so applied oxygen and checked blood test. Blood glucose was 16 mg/dl. The evaluation of insulin/glucose ratio was performed glucose 34 mg/dl (normal range: 60108 mg/dl), insulin 67.43 μIU/ml (normal range: 1225 μIU/ml), insulin/glucose ratio 1.98. Abdominal USG was performed to find pancreas anomaly, there were normal pancreas and intrahepatic portosystemic shunt. Liver dynamic CT showed intrahepatic portosystemic shunt middle and left hepatic vein between left portal vein and hypoplasia of right portal vein. The patient was treated with intravenous 10% glucose (glucose IR 17 mg/kg per min) and frequent oral feeding, hypoglycemia slightly improved. So with frequent feeding and glucose monitoring at home, we have plan to have surgical correction.
Discussion: Even though rare, congenital portosystemic shunt can cause hyperinsulinism and hypoglycaemia in infant, so imaging study is needed about vasculature in liver with pancreas, avoiding unnecessary treatment, for example, diazoxide.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology