There are two types of this inherited condition, glycogen storage disease IXa1 and glycogen storage disease IXa2 that affect the liver of an individual.[6] Mutations in
PHKA2 have been seen in individuals with glycogen storage disease IXa2.[medical citation needed]
Management
The management of Glycogen storage disease IX requires treatment of symptoms by frequent intake of complex carbohydrates and protein to combat the low blood sugar. A nutritionist will advise on suitable diets. Liver function is regularly monitored and problems managed as they arise. However, liver problems have only been successfully treated by a
transplant. Routine checks of metabolism are needed to ensure blood sugar (glucose) and ketones are managed. Regular moderate exercise is beneficial, although over-vigorous exercise is to be avoided, especially in those with enlarged livers.[1][7]
Albash, Buthainah; Imtiaz, Faiqa; Al-Zaidan, Hamad; Al-Manea, Hadeel; Banemai, Mohammed; Allam, R.; Al-Suheel, Ali; Al-Owain, Mohammed (2014). "Novel PHKG2 mutation causing GSD IX with prominent liver disease: report of three cases and review of literature". European Journal of Pediatrics. 173 (5): 647–653.
doi:
10.1007/s00431-013-2223-0.
ISSN1432-1076.
PMID24326380.
S2CID37564174.
Stoler, edited by Raymond R. Tubbs, Mark H.; Stoler, Mark H. (2009).
Cell and tissue based molecular pathology (1st ed.). Philadelphia: Churchill Livingstone/Elsevier.
ISBN978-1437719482. Retrieved 6 December 2017. {{
cite book}}: |first1= has generic name (
help)CS1 maint: multiple names: authors list (
link)