Low-dose diazoxide is safe and effective in infants with transient hyperinsulinism

Clin Endocrinol (Oxf). 2024 Feb;100(2):132-137. doi: 10.1111/cen.14987. Epub 2023 Dec 7.

Abstract

Objective: Transient hyperinsulinism (THI) is the most common form of recurrent hypoglycaemia in neonates beyond the first week of life. Although self-resolving, treatment can be required. Consensus guidelines recommend the lower end of the diazoxide 5-15 mg/kg/day range in THI to reduce the risk of adverse events. We sought to determine if doses <5 mg/kg/day of diazoxide can be effective in THI.

Design, patients, measurments: Infants with THI (duration <6 months) were treated with low-dose diazoxide from October 2015 to February 2021. Dosing was based on weight at diazoxide start: 2 mg/kg/day in infants 1000-2000 g (cohort 1), 3 mg/kg/day in those 2000-3500 g (cohort 2) and 5 mg/kg/day in those >3500 g.

Results: A total of 73 infants with THI (77% male, 33% preterm, 52% small-for-gestational age) were commenced on diazoxide at a median age of 11 days (range 3-43) for a median duration of 4 months (0.3-6.8), with no difference between cohorts. The mean effective diazoxide dose was 3 mg/kg/day (range 1.5-10); 35% (26/73) required an increase from their starting dose, including 60% (9/15) of cohort 1. There was no association between perinatal stress risk factors or treatment-related characteristics and dose increase. Adverse events occurred in 13 patients (18%); oedema (12%) and hyponatraemia (5%) were the most common. Two infants developed suspected necrotising enterocolitis (NEC); none had pulmonary hypertension.

Conclusion: Diazoxide doses <5 mg/kg/day are effective in THI. While the nature of the association between diazoxide and NEC was unclear, other adverse events were mild. We suggest considering starting doses as low as 2-3 mg/kg/day in THI to balance the side effect risk while maintaining euglycaemia.

Keywords: diazoxide; diazoxide adverse events; hyperinsulinism; hypoglycaemia treatment.

MeSH terms

  • Congenital Hyperinsulinism* / drug therapy
  • Diazoxide / adverse effects
  • Female
  • Humans
  • Hyperinsulinism* / drug therapy
  • Hypoglycemia* / drug therapy
  • Infant
  • Infant, Newborn
  • Infant, Small for Gestational Age
  • Male
  • Risk Factors

Substances

  • Diazoxide