IMPE Abstracts

Background: Congenital hyperinsulinism (CHI) is characterized by hyperinsulinemic hypoglycemia, conferring a high risk of neurodevelopmental impairment if not treated promptly and sufficiently. The conventional medications are often ineffective and while some forms of CHI are curable by surgery, new treatment modalities are advocated. Glucagon counteracts the action of insulin, but the use in CHI has been limited. We aimed to evaluate the efficacy and side effects of continuous intravenous glucagon in the treatment of CHI at our institution.

Methods: Retrospective single-center cohort study on patients with CHI treated with continuous glucagon infusion.

Results: Of 132 patients with CHI, 21 (16%) recieved continuous IV glucagon. The median (range) age at glucagon treatment was 48 (3;871) days; glucagon dose 2.5 (0.3;22.1) µg/kg/hr; duration of treatment 7 (1;58) days. Concomitant treatment included IV glucose (n=21), octreotide (n=16), and diazoxide (n=10). Glucose infusion rate (GIR) was reduced within 24 hours of glucagon treatment, -5.2 (-13.5;+1.6) mg/kg/min (P<0.0001). The number of hypoglycemia episodes ±4 days from glucagon onset was unchanged 0 (-15;+9). Potential side effects included hyperglycemia (n=19), vomiting (n=10), skin rash (n=10) and distress (n=9), although causality could not be established. One patient developed necrolytic migratory erythema (NME) after 58 days of glucagon treatment with an exceptionally high accumulated glucagon dose of 10,336 µg/kg.

Conclusions: Continuous intravenous glucagon significantly reduced GIR within 24 hours. Excess glucagon dosing may lead to NME. The role of glucagon products in the treatment of CHI remains to be determined.