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Table 3 Management of pathological KH in children

From: Towards enhanced understanding of idiopathic ketotic hypoglycemia: a literature review and introduction of the patient organization, Ketotic Hypoglycemia International


Range of use



 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





  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




  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



  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

  1. KH ketotic hypoglycemia, PG plasma glucose
  2. *Continuous glucose monitoring cannot stand alone due to inaccuracy at low glucose concentrations, and needs further research specifically in KH