PERSISTENT HYPERINSULINAEMIC HYPOGLYCAEMIA OF INFANCY: CASE REPORT

Hyperinsulinism, although rare, is the most common cause of persistent hyperinsulinaemichypoglycaemia in infancy. Because of persistent hypoglycaemia, serious difficulties areencountered in the long term management of this condition. A male neonate, after anuncomplicated full-term pregnancy, had been admitted to another hospital withconvulsions on the third post-natal day. Meningitis had been suspected at that timeand treated with phenobarbital and he had been discharged from the hospital. At threemonthsold he was referred to our department for persistent convulsions and lethargy.His parents were of 1st degree consanguinity. His blood glucose level was found to be24 mg/dl (1.33 mmol/L). Because of the dangerously high insulin level during hypoglycaemia(insulin/glucose >0.3), the absence of ketonuria, and the need for a high dose of glucoseinfusion (> 15 mg/kg/min) to achieve normoglycaemia and a glycaemic response toglucagon despite the hypoglycaemia, a diagnosis of persistent hyperinsulinaemichypoglycaemia of infancy was made. Since maximal doses of prednisone, glucagon,diazoxide, octreotide and high infusion of glucose were ineffective in achievingnormoglycaemia, a subtotal (80%) pancreatectomy was done. Postoperatively intermittenthypoglycaemic episodes continued. These were controlled with low doses of octreotide.Histology revealed diffuse adenomatous hyperplasia (nesidoblastosis). The boy is nowin the sixth post-operative month and developing normally