Hyperinsulinemic Hypoglycemia in Patients with Intestinal Failure Receiving Parenteral Nutrition with a High Glucose Infusion Rate

Abstract

Management of persistent hyperinsulinemic hypoglycemia in infant and neonatal patients remains controversial. Prolonged hyperinsulinemic hypoglycemia is generally responsive to a high glucose infusion rate (GIR), however the long term adverse effects of providing a high GIR are not well described in the literature. Patients with intestinal failure who develop parenteral nutrition associated liver disease also require a high GIR to meet nutrition needs during lipid minimization. Here we present two cases of confirmed hyperinsulinemic hypoglycemia that developed in pediatric patients with intestinal failure who received a high GIR due to lipid minimization. Our first patient presented to our clinic as a parenteral nutrition dependent four year old female with a history of gastroschisis and jejunal atresia with resultant short bowel syndrome (based on outside records, she was noted to have lost 87% of her small bowel but had 2/3 of her colon) and chronic kidney disease. Her parenteral nutrition utilized lipid minimization, providing her with 1 g/kg/day of lipids and a GIR of 14.1 mg/kg/min. She was diagnosed with hyperinsulinemic hypoglycemia and was started on treatment with diazoxide. Our second patient presented to the intensive care nursery as a three month old female with a history of type IIIB ileal atresia (status post repair on day 3 of life) and failure to thrive. Subsequently, she had multiple bowel resections with lysis of adhesions and a right hemicolectomy, with 63 centimeters of remaining small bowel noted. In the intensive care nursery, she was receiving lipid minimization with a GIR of 26.25 mg/kg/min and 1 g/kg/day of lipids. She was diagnosed with hyperinsulinemic hypoglycemia and was started on treatment with diazoxide. Non-genetic risk factors for hyperinsulinemic hypoglycemia are not well established. From these cases, we postulate that providing high amounts of glucose through a high GIR during lipid minimization in parenteral nutrition alters the pancreatic beta cell regulatory mechanism for insulin release, leading to hyperinsulinism. To our knowledge, this is the first report of this association in the literature in the intestinal failure patient population.