Priority 10 from the Liver Glycogen Storage Disease (International) PSP

UNCERTAINTY: What are the risks and benefits of different options for overnight treatment for patients with liver Glycogen Storage Disease and how can we maximize safety?  (JLA PSP Priority 10)
Overall ranking 10
JLA question ID 0090/10
Explanatory note

Overnight treatment for liver GSD can consist of different modalities such as cornstarch therapy or continuous gastric dripfeeding. Complications of these treatment modalities have been reported in literature, but there is a lack of larger cohorts describing risks and benefits of these modalities.


For details of the evidence checked, please see the spreadsheet held on the JLA website.

Health Research Classification System category Metabolic and endocrine Patient, carer and healthcare professionals
Extra information provided by this PSP
Original uncertainty examples Is nighttime feeding via tube necessary? ~ Is there proven evidence that over night cornstarch started in infancy has no worse consequences in terms of glucose responsivenes and secondary complications in GSD1a compared to overnight gastric trip feeding? ~ Over night Blood Sugar management. ~ How can  safety be improved during the night? ~ Risk of dislocation of the tube in GSD 1 and 3 during the night. How to secure this without burdening the parents too much?
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