Idiopathic ketotic hypoglycemia

Ketotic hypoglycemia (KH) Ketotic hypoglycemia can be seen in children because of growth hormone deficiency, cortisol deficiency, or metabolic diseases with intact fatty acid consumption, including glycogen storage diseases (glycogenosis; GSD) type 0, III, VI, and IX. When these diagnoses are excluded, ketotic hypoglycemia can be categorized as unexplained or idiopathic (IKH), otherwise known as accelerated starvation. The primary treatment is dietary prevention with long carbohydrates (e.g. uncooked cornstarch) and frequent meals. In some more severely affected patients, continuous feeding may be needed through a gastrostomy tube. Emergency treatment constitutes of oral or i.v. glucose, eventually i.m. glucagon, to raise plasma glucose, which will prevent further lipolysis. However, ketones can take hours to be eliminated. In more severely affected patients, the ketone production can be significant before hypoglycemia occurs, why recognition of increased ketones above approximately 1.5 mol/L is important, sometimes already when the glucose concentration goes below 3.9 mmol/L (70 mg/dL). 
 
Idiopathic ketotic hypoglycemia is the most frequent state with low blood sugar in preschool kids. However, the incidence is unknown. Most commonly it debuts around 18 months with spontaneous normalization around 6-7 years of age, but this does not account for all. The severity and frequency of the hypoglycemia span wide, but IKH is usually associated with relatively mild hypoglycemia, where p-glucose rarely goes below 2,0 mmol/L. the frequency varies from multiple daily to single episodes with fever, vomiting and/or diarrhea. IKH is by some acknowledged as normal physiological variation right below the normal range for fasting tolerance (the lower tail of the Gaussian distribution). GSD variants, special GSD type IXa can, however, be mistakenly diagnosed as IKH 
 
Source: Professor Henrik Christesen, Odense University Hospital, Denmark. Henrik is head of the Ketotic Hypoglycemia International Scientific Advisory Board. 

The Danish hypoglycemia expert

Current titles and working place: Consultant in Paediatrics, clinical professor, MD, PhD. Hans Christian Andersen Children’s Hospital, Odense University Hospital, Denmark. 

This patien union was created on the basis of the support and knowledge of Henrik Christesen, where he now sits as head of the Scientific Advisory Board in Ketotitc Hypoglycemia International. Here is a list of just a few of his accomplishment:

  • 97 peer-reviewed papers
  • 83 press/popular presentations
  • 2 international book chapters 
  • 2 national guidelines (neonatal hypoglycemia/hyperinsulinism and vitamin D intoxication)
  • Chair of the Danish Study Group of Paediatric Endocrinology 
  • European Society of Paediatric Endocrinology ESPE, Research Unit Award for international research in Congenital Hyperinsulinism, Sept. 2012
  • Most cited paper 2013, Acta Obstetrica et Gynecologica Scandinavia awatd, 39th Nordic Congress of Obstetrics and Gynecology, 10-12/6 2014, Stockholm, Sweden
  • Principal supervisor for 6 PhD students