The precise prevalence of GSDVI is unknown. No less than eleven instances have been reported in the medical literature, although this condition is likely to be underdiagnosed as a result of it may be tough to detect in youngsters with mild signs or adults with no symptoms. GSDVI is extra common within the Old Older Mennonite inhabitants, with an estimated incidence of 1 in 1,000 individuals. Mutations in the PYGL gene trigger GSDVI. The PYGL gene offers instructions for making an enzyme known as liver glycogen phosphorylase. This enzyme is discovered solely in liver cells, where it breaks down glycogen into a sort of sugar known as glucose-1-phosphate. Additional steps convert glucose-1-phosphate into glucose, a easy sugar that is the main energy supply for many cells within the physique. PYGL gene mutations forestall liver glycogen phosphorylase from breaking down glycogen successfully. Because liver cells can't break down glycogen into glucose, individuals with GSDVI can have hypoglycemia and may use fats for Healthy Flow Blood product power, resulting in ketosis.