Priority 19 (Joint) from the Liver Glycogen Storage Disease (International) PSP

UNCERTAINTY: When should liver transplantation be considered in patients with liver Glycogen Storage Disease and what are the (dis)advantages and long-term outcomes? (JLA PSP Priority 19 (Joint))
Overall ranking 19
JLA question ID 0090/19 (Joint)
Explanatory note

Liver transplantation is a modality of treatment that is indicated for specific situations for patients with liver GSD. However, not much follow-up information on long-term outcomes are known. 


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 When to consider liver or transplantation for best outcomes? ~ The role and timing of livertransplant in the treatment of GSDI/GSDIII. ~ When is transplant indicated in the absence of significant fibrosis or a tumor? ~ Particular care of these patients in the pre- and post-liver transplantation 
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