Table 1.

Main endocrinologic causes of hypoglycemia. Abbreviations: M.A.: metabolic acidosis. The up arrow indicates an increased synthesis or action of the respective element. The down arrow indicates a deficiency in synthesis or action of the respective element.

	Type	Causes	Clinical Features	Biochemical Features	Treatment	Potential New Therapies
	Hyperinsulinism	Insulin	Hypoglycemic symptoms	Hypoglycemia, increased insulin and C-peptide, normal ketones and fatty acids, absence of M.A., positive glycemic response to glucagon	Diazoxide, somatostatin analogs (octreotide), long-acting release somatostatin analogs (lanreotide)	Glucagon-like peptide-1 receptor antagonists, pharmacological chaperones
	GH deficiency	GH	Hypoglycemic symptoms, prolonged and recurrent jaundice, growth deceleration	Hypoglycemia, increased ketones, fasting M.A.	Hormone replacement therapy
Endocrine disorders	Adrenal insufficiency	Cortisol	Hypoglycemic symptoms, cholestasis	Fasting hypoglycemia, increased ketones, fasting M.A.	Hormone replacement therapy
	Pediatric neoplastic formations (Wilms’ tumor, nephroblastoma, lymphomas/leukemias)	IGF-II	Hypoglycemic symptoms	Fasting hypoglycemia and postprandial hyperglycemia, suppressed insulin secretion, low ketones and fatty acids, suppressed glucagon and GH release	Surgical treatment, glucocorticoids and GH administration	Anti-IGF-II monoclonal antibodies