Oral beta-hydroxybutyrate supplementation in two patients with hyperinsulinemic hypoglycemia: monitoring of beta-hydroxybutyrate levels in blood and cerebrospinal fluid, and in the brain by in vivo magnetic resonance spectroscopy

This report describes two 6-month-old infants with persistent hyperinsulinemic hypoglycemia who received oral DL sodium beta-hydroxybutyrate for 5 and 7 months while on frequent tube-feedings and octreotide. At 880 to 1000 mg/kg per day, blood beta-OHB reached levels comparable to a 16 to 24 hour fast, and cerebrospinal fluid levels after single doses of 4 and 8 g reached levels comparable to a 24 to 40 hour fast. High beta-OHB to acetoacetate ratios indicated an exogenous origin, and proton magnetic resonance spectroscopy showed increased intracerebral beta-OHB. The supplement was tolerated without side effects.