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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
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
  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