Glycogen storage disease type I secondary prevention: Difference between revisions
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==Secondary Prevention== | ==Secondary Prevention== | ||
Effective measures for secondary prevention of GSD type 1 include: | Effective measures for secondary prevention of GSD type 1 include:<ref name="KishnaniAustin2014">{{cite journal|last1=Kishnani|first1=Priya S.|last2=Austin|first2=Stephanie L.|last3=Abdenur|first3=Jose E.|last4=Arn|first4=Pamela|last5=Bali|first5=Deeksha S.|last6=Boney|first6=Anne|last7=Chung|first7=Wendy K.|last8=Dagli|first8=Aditi I.|last9=Dale|first9=David|last10=Koeberl|first10=Dwight|last11=Somers|first11=Michael J.|last12=Burns Wechsler|first12=Stephanie|last13=Weinstein|first13=David A.|last14=Wolfsdorf|first14=Joseph I.|last15=Watson|first15=Michael S.|title=Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics|journal=Genetics in Medicine|year=2014|issn=1098-3600|doi=10.1038/gim.2014.128}}</ref> | ||
* Blood glucose (BG) monitoring | |||
* Gastrointestinal or Nutritional recommendations | |||
===Blood glucose (BG) monitoring=== | |||
*Initial diet prescription is established on the basis of frequent BG monitoring. Afterwards, BG monitoring is done randomly to avoid asymptomatic hypoglycemia. | |||
*Documentation of BG testing is done before each clinic visit to adjust diet, CS intake, and overnight gastric feedings (OGFs). | |||
The following BG levels should be checked for 2–3 days before the clinic visit: | |||
**Before meals | |||
**Before cornstarch (CS) intake | |||
**Before and after exercise | |||
*If the cornstarch dose is changed, BG levels should be checked after 4 hours and then at hourly intervals to establish the duration of effectiveness. Effectiveness is measured by the duration of time for which the dose of CS will maintain the BG level >70 mg/dl. | |||
====Lactate meter==== | |||
The lactate meter may be a good supplement to glucose monitoring, especially during times of illness to help prevent acute deterioration, to avoid hospitalization, or to alert the parent that is time to go to the emergency room. Th | |||
{| | {| | ||
| style="background:#4479BA; color: #FFFFFF;" + |'''Gastrointestinal | | style="background:#4479BA; color: #FFFFFF;" + |'''Gastrointestinal or Nutritional recommendations''' | ||
|- | |- | ||
| style="background:#F5F5F5;" + | | | style="background:#F5F5F5;" + | |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Secondary Prevention
Effective measures for secondary prevention of GSD type 1 include:[1]
- Blood glucose (BG) monitoring
- Gastrointestinal or Nutritional recommendations
Blood glucose (BG) monitoring
- Initial diet prescription is established on the basis of frequent BG monitoring. Afterwards, BG monitoring is done randomly to avoid asymptomatic hypoglycemia.
- Documentation of BG testing is done before each clinic visit to adjust diet, CS intake, and overnight gastric feedings (OGFs).
The following BG levels should be checked for 2–3 days before the clinic visit:
- Before meals
- Before cornstarch (CS) intake
- Before and after exercise
- If the cornstarch dose is changed, BG levels should be checked after 4 hours and then at hourly intervals to establish the duration of effectiveness. Effectiveness is measured by the duration of time for which the dose of CS will maintain the BG level >70 mg/dl.
Lactate meter
The lactate meter may be a good supplement to glucose monitoring, especially during times of illness to help prevent acute deterioration, to avoid hospitalization, or to alert the parent that is time to go to the emergency room. Th
Gastrointestinal or Nutritional recommendations |
|
Adopted from Genetics in medicine[1] |
References
- ↑ 1.0 1.1 Kishnani, Priya S.; Austin, Stephanie L.; Abdenur, Jose E.; Arn, Pamela; Bali, Deeksha S.; Boney, Anne; Chung, Wendy K.; Dagli, Aditi I.; Dale, David; Koeberl, Dwight; Somers, Michael J.; Burns Wechsler, Stephanie; Weinstein, David A.; Wolfsdorf, Joseph I.; Watson, Michael S. (2014). "Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics". Genetics in Medicine. doi:10.1038/gim.2014.128. ISSN 1098-3600.