Priority 3 from the Liver Glycogen Storage Disease (International) PSP
|UNCERTAINTY: How should optimal metabolic control both clinically and biochemically (like lactate, ketones and/or lipids) be achieved in liver Glycogen Storage Disease? (JLA PSP Priority 3)|
|JLA question ID||0090/3|
The goal of treatment in liver GSD is to prevent metabolic derangement. There are several manners in which metabolic derangement can be prevented, such as dietary treatment.
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 important is it to keep ketone levels to a minimum? ~ prognostic markers for type IX? ~ What biochemical parameters are useful in the patient with GSD? How do you monitor the treatment in the patient to observe its effectiveness, as well as how to make appropiate adjustments in the treatment? ~ Why are there remaining strongly deviant metabolic parameters (lactate/triglycerides) even if there are no hypoglycemia's?|
|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|