Glycogen storage disease type I secondary prevention: Difference between revisions
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{{CMG}}; {{AE}} | {{CMG}}; {{AE}} | ||
==Overview== | ==Overview== | ||
==Secondary Prevention== | ==Secondary Prevention== | ||
Effective measures for secondary prevention of GSD type 1 include: | |||
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! style="background:#4479BA; color: #FFFFFF;" align="center" + |Gastrointestinal /Nutritional recommendations | |||
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| style="background:#F5F5F5;" + | | |||
*A metabolic dietician is an important member of the team. If not available, one should be consulted. | |||
*Maintaining blood glucose levels ≥70 mg/dl is important to achieve good metabolic control. Levels should e kept consistent to avoid hypoglycemia and fluctuations blood glucose levels. | |||
*In infants and children: | |||
**Avoid fasting for more than 3-4 hours. | |||
**Offer small, frequent feedings; avoid or limit sucrose, fructose, and galactose (a soy formula such as Prosobee may be used overnight). | |||
**Access via NG or G-tube placement is recommended for emergencies and/or for OGFs; caution with surgical G-tube placement should be taken in GSD 1b. | |||
**Monitor blood glucose before feeds. | |||
**Raw, uncooked cornstarch may be introduced between 6 and 12 months of age/ | |||
**Continuous gastric feedings may be used overnight. | |||
*In adolescent and adults: | |||
**Avoid fasting for more than 5-6 hours with the use of raw, uncooked cornstarch and/or OGFs; it is important to not change the brand of cornstarch. If changed, then monitoring of BG levels after the change is necessary. | |||
**Plan for small, frequent meals (nutrient distribution:60-70% carbohydrates, 10-15% protein, <30% fat); avoid or limit sucrose, fructose, and galactose. | |||
**Regular blood glucose monitoring is needed, especially during periods of growth. | |||
*Multivitamins, calcium, and vitamin D are necessary because of the restricted nature of the diet. | |||
*Both overtreatment and undertreatment are harmful. Overtreatment can result in insulin resistance. | |||
*'''Good glucose control improves several of the metabolic sequelae of GSD 1.''' | |||
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| style="background:#DCDCDC; + |<small>'''Adopted from [[https://www.nature.com/gim/journal/vaop/ncurrent/fig_tab/gim2014128b2.html| Genetics in medicine]]'''</small><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> | |||
|} | |||
==References== | ==References== | ||
Revision as of 15:35, 6 November 2017
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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:
| Gastrointestinal /Nutritional recommendations |
|---|
|
| Adopted from [Genetics in medicine][1] |
References
- ↑ 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.