In the affected boy, KH seems to be the result of a reduced capacity to use ketone bodies, leading to increased peripheral metabolism of glucose that cannot be met by hepatic glucose production. Because the boys are homozygotic twins and only one of them is affected, the ketotic hypoglycemia is most likely caused by an altered imprinting of gene(s) involved in regulating metabolic pathways.
Metabolism of glucose to lactate and glycerol in subcutaneous adipose tissue is a rapid process that can be monitored in vivo by administration of stable isotope labelled glucose into the microdialysis probe. In adults at rest about one-fourth of adipose tissue lactate is produced locally.
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