Pathological forms of KH are seen in children with deficiencies in the counter regulatory hormones such as cortisol or growth hormone, or in metabolic conditions such as glycogen storage disease (GSD, specifically GSD 0, III, VI, and IX), disorders of ketone transport and ketone utilization, and defects in protein metabolism, Table 1 [30, 31]. This evidence concerns the gene GH1 and disorder of glycogen metabolism.