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. |
| Evidence |
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 |