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.