title: Persistent hyperinsulinaemic hypoglycaemia of infancy: case report.
abstract: Hyperinsulinism, although rare, is the most common cause of persistent hyperinsulinaemic hypoglycaemia in infancy. Because of persistent hypoglycaemia, serious difficulties are encountered in the long term management of this condition. A male neonate, after an uncomplicated full-term pregnancy, had been admitted to another hospital with convulsions on the third post-natal day. Meningitis had been suspected at that time and treated with phenobarbital and he had been discharged from the hospital. At three-months old 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 be 24 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 glucose infusion (> 15 mg/kg/min) to achieve normoglycaemia and a glycaemic response to glucagon despite the hypoglycaemia, a diagnosis of persistent hyperinsulinaemic hypoglycaemia of infancy was made. Since maximal doses of prednisone, glucagon, diazoxide, octreotide and high infusion of glucose were ineffective in achieving normoglycaemia, a subtotal (80%) pancreatectomy was done. Postoperatively intermittent hypoglycaemic episodes continued. These were controlled with low doses of octreotide. Histology revealed diffuse adenomatous hyperplasia (nesidoblastosis). The boy is now in the sixth post-operative month and developing normally.
authors.affiliation: Department of Paediatrics, Pamukkale University, School of Medicine, Denizli, Turkey.
authors.forename: S
authors.initials: S
authors.lastname: Semiz
authors.forename: I
authors.initials: I
authors.lastname: Bircan
authors.forename: S
authors.initials: S
authors.lastname: Akçurin
authors.forename: E
authors.initials: E
authors.lastname: Mihçi
authors.forename: M
authors.initials: M
authors.lastname: Melikoglu
authors.forename: G
authors.initials: G
authors.lastname: Karagüzel
authors.forename: B
authors.initials: B
authors.lastname: Kiliçaslan
authors.forename: G
authors.initials: G
authors.lastname: Karpuzoğlu
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001786:Blood Glucose / Q000032:analysis; D003241:Consanguinity; D003981:Diazoxide / Q000627:therapeutic use; D004827:Epilepsy / Q000209:etiology; D005934:Glucagon / Q000627:therapeutic use; D006801:Humans; D006946:Hyperinsulinism / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy*; D007003:Hypoglycemia / Q000209:etiology*; D007231:Infant, Newborn; D007328:Insulin / Q000097:blood; D008297:Male; D015282:Octreotide / Q000627:therapeutic use; D010180:Pancreatectomy / Q000379:methods; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D001786:Blood Glucose; D007328:Insulin; D005934:Glucagon; D003981:Diazoxide; D015282:Octreotide; D011241:Prednisone