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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 threemonths
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.
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 threemonths
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.
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