Priority 15 from the Liver Glycogen Storage Disease (International) PSP

UNCERTAINTY: What are the effects of different kinds of Ketogenic Diet in patients with Glycogen Storage Disease Type III? (JLA PSP Priority 15)
Overall ranking 15
JLA question ID 0090/15
Explanatory note

There is much debate in literature on the indication and effect of ketogenic diet for patients which liver GSD. This question illustrates that more research is needed into the treatment effects.

Evidence

For details of the evidence checked, please see the spreadsheet held on the JLA website.

Health Research Classification System category Metabolic and endocrine
Extra information provided by this PSP
Original uncertainty examples Is the ketogenic diet/modified Atkins diet a useful therapy option for GSD type III? ~ Place of a ketogenic diet and above all how to accept such a diet in the social life in the long term ~ Do ketogenic diets help cardiomyopathy in GSD 111?
Submitted by Patient, carer and healthcare professionals
PSP information
PSP unique ID 0090
PSP name International Glycogen Storage Disease PSP
Total number of uncertainties identified by this PSP 72 (To see a full list of all uncertainties identified, please see the detailed spreadsheet held on the JLA website)
Date of priority setting workshop 24 May 2019