True increase in islet cella) Nesidioblastomab) Type II DMc) Insulinomad) Pancreatitis
Also known as persistent hyperinsulinemic hypoglycemia of infancy. Characterized by diffuse hyperfunction of pancreatic beta cells with enlargement of their nuclei. Neither the beta cell proliferation rate nor the overall beta cell mass is increased.
Clinical Features

Early recognition of congenital hyperinsulinism is critical because, if untreated, profound hypoglycemia may lead to brain damage.
Babies may be described as jittery, floppy, or lethargic; seizures are common.

Diagnosis

Inappropriately elevated insulin in the setting of hypoglycemia, along with the need for continuous glucose infusion (<15 mg/kg/min) to maintain normoglycemia confirms the diagnosis.
Pancreatic venous sampling is used to make the diagnosis.

Treatment

Continuous glucose administration with suppression of insulin secretion by diazoxide or somatostatin.
Operation is necessary in more than two thirds of cases.
Treatment consists of near total (95-98%) pancreatectomy.
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