We appreciate the discussion by Hussain concerning our article on the administration of DL sodium β-hydroxybutyrate in two cases with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) as published in Pediatric Research (1).

The idea behind administration of DL sodium β-hydroxybutyrate was to provide an alternative energy fuel to protect the brain from damage potentially caused by recurrent hypoglycemic events. This is a neuroprotective approach, which is not expected to alter the natural history of hyperinsulinism. Therefore, with respect to insulin secretion and glucose homeostasis, preexisting treatment with octreotide and feeding schedules remained unchanged over the whole observational period.

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