Priority 6 from the Liver Glycogen Storage Disease (International) PSP

UNCERTAINTY: How can existing cornstarch preperations be modified or alternative treatments be implemented that are easier to administer and/or keep blood sugar levels more stable for patients with liver Glycogen Storage Disease? (JLA PSP Priority 6)
Overall ranking 6
JLA question ID 0090/6
Explanatory note

The goal of cornstarch therapy for liver GSD is the maintanance of glucose homeostasis to prevent acute metabolic derangement leading to short-term and long-term complications, but the therapy be difficult to administer and does not provide a solution to every situation. Research of this summary question should focus on finding alternative preparations or treatments that are easier to administer or keep blood glucose levels more stable. 


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 Could exist a pill to be in the stomach and give slowly carbs in order to be easier to take a big dose of cornstarch? ~ Making long lasting cornstarch that is more palpable and less volume? ~ what are the differences in efficacy of various food starches (rice vs. potato vs. wheat vs corn vs. cassava-tapioca) providing carbs for GSD1 pts? ~ starting infants on cornstarch and the transition from continuous feeds to UCS ~ Are there any studies regarding the use of glycoside during the day? 
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