Table 2 Diagnostic criteria for patients with HH [1, 101]

Diagnostic criteria

• Plasma glucose <3 mmol/l with:

• Detectable serum insulin

• Detectable C-peptide (In endogenous HH)

• Suppressed/low serum ketone bodies

• Suppressed/low serum fatty acids

Supportive evidences (when diagnosis is in doubt or difficult):

• Glucose infusion rate > 8 mg/kg/min

• Positive glycaemic (>1.5 mmol/L) response to intramuscular/ intravenous glucagon

• Positive glycaemic response to a subcutaneous/intravenous dose of octreotide

• Low serum levels of IGFBP1 [insulin negatively regulates the expression of IGFBP1]

• Suppressed branch chain (leucine, isoleucine and valine) amino acids

• Provocation tests (leucine loading or exercise testing) may be needed in some patients

• Supressed/undetectable urine ketone

• Normal lactic acid

• Normal plasma hydroxybutyrylcarnitine^a

• Normal ammonia^b

• Appropriate counterregulatory hormone response^c

 -Cortisol > 20 mcg/dl (500 nmol/l)

 -Growth hormone > 7 ng/ml

 In the neonatal period if the hypoglycaemia persists for >48 h this will require investigation