Table 5.

Diagnostic criteria of CH.

Cardinal diagnostic criteria	Low plasma glucose (<3 mmol/L) with
	Detectable serum insulin
	Detectable C–peptide
Biochemical criteria	Suppressed/low beta–hydroxybutyrate and acetoacetate
	Suppressed/low serum free fatty acid
Clinical criteria	Increased requirement of glucose infusion rate (>8 mg/kg/min)
	Positive response to i.m./i.v. glucagon (glycemic response >1.5 mmol/L)
Supportive criteria	Positive response to s.c./i.v. octreotide
	Low serum levels of IGFBP–1 (suppressed by insulin)
	Suppressed branch chain amino acids
	Normal lactic acid
	Normal plasma hydroxybutyrylcarnitine
	Normal ammonia
	Appropriate counterregulatory hormone response (cortisol >20 mcg/dl, GH > 7 ng/ml)
	Provocation test (leucine loading or exercise testing) may be needed in some patients

Modified from Vora et al. (43).