Priority 16 from the Liver Glycogen Storage Disease (International) PSP
|UNCERTAINTY: What is the needed restriction of lactose, fructose or saccharose in different types of liver Glycogen Storage Disease? (JLA PSP Priority 16)|
|JLA question ID||0090/16|
Guidelines emphasize that lactose, fructose and saccharose are carbohydrates that are have to be strictly avoided in GSD Type I.
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||I would like to know retriction of fructose and galactose restriction. What is the best way to go? ~ Can my son have limited amounts of lactose and fructose? I get different answers depending who I speak to. He is type 1A. ~ Can we clear the diet from adolescence concerning lactose and fructose without risk.|
|Submitted by||Patient, carer and healthcare professionals|
|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|