Priority 17 from the Liver Glycogen Storage Disease (International) PSP

UNCERTAINTY: How can patients with liver Glycogen Storage Disease achieve and/or maintain a healthy weight throughout life?  (JLA PSP Priority 17)
Overall ranking 17
JLA question ID 0090/17
Explanatory note

Weight gain can be a complication of liver GSD since the cornerstone of treatment is a diet rich in complex carbohydrates. This question emphasized that more research is necessary on protocols to prevent, achieve or maintain a healthy weight, without metabolic derangement occuring due to undertreatment. 
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 Development of overweight and diabetes at an adult age. ~ The weight gain that comes with consuming cornstarch ~ Is there a possibility to lose weight despite the high carbohydrates?  
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