ABSTRACT
Persistent hyperinsulinemic hyperglycemia of infancy
(PHHI), or congenital hyperinsulinism (CHI) as it is now
more broadly termed, is a spectrum of conditions character-
ized by profound hypoglycemia in the presence of inappro-
priately high insulin secretion.1,2 Although rare overall, CHI
is a significant cause of hypoglycemic brain injury and mental
retardation3 and therefore, all those involved in the medical
and surgical management of neonates need to be familiar
with this condition so that the diagnosis can be made
promptly, and early treatment implemented. This chapter
provides an overview of the etiology, diagnosis, and manage-
ment of CHI to facilitate this.