Table 3.

Management of pathological KH in children

	Range of use
Monitoring
Bedside glucometer and blood ketones	Initial work up
	Fever, vomiting or diarrhea; at symptoms/ morning fasting values/ frequent daily
Continuous glucose monitoring*	Initial work up; for months or years in more severe, pathological KH
Prevention
Dietary
Complex carbohydrates, protein	Before sleep only; for every meal
Meal interval	Dependent on age, severity and frequency of KH
Uncooked corn starch	½–1 (− 2) g/kg, 1–4 × daily
Long-release corn starch	Severe, frequent KH
Continuous gastrostomy tube feeding	Severe, frequent KH. Night feeding with maltose 1/2–1 g/kg/h; other tube feeding products
Acute treatment
Dietary
Sugar-rich drinks and food	KH attacks without compromised swallowing. Add complex carbohydrates, eventual protein
Buccal carbohydrate gel application	KH attacks with compromised swallowing. 1/2–1 tube, eventually repeated
Medication
I.m. glucagon	Severe KH attacks with unconsciousness. 30–40 mcg/kg, maximal 1 mg. Only if proven efficient and safe at specialist center
I.v. glucose or dextrose	Severe KH attack. Ensure PG > 3.9 mmol/L (70 mg/dL). Continue until blood ketones < 0.5 mmol/L

KH ketotic hypoglycemia, PG plasma glucose

*Continuous glucose monitoring cannot stand alone due to inaccuracy at low glucose concentrations, and needs further research specifically in KH