Priority 5 from the Liver Glycogen Storage Disease (International) PSP

UNCERTAINTY: What is the best way to start dietary treatment, finding the optimal doses, and to administer the diet for patients with liver Glycogen Storage Disease? (JLA PSP Priority 5)
Overall ranking 5
JLA question ID 0090/5
Explanatory note

Dietary treatment for patients with liver GSD can be initiated via several options, depending on internal and external factors of the individual patient. 


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 How frequently to recommend an in-hospital stay for adjustment of diet and cornstarch? ~ How best to administer cornstarch, timing with respect to meals? ~ Can the use of the gastric tube be forever? 
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