Hypovitaminosis D in glycogen storage disease type I.
Journal Article (Journal Article)
Glycogen storage disease type I (GSD I) is caused by inherited defects of the glucose 6-phosphatase complex, resulting in fasting hypoglycemia, lactic acidosis, hyperuricemia and hyperlipidemia. Sixteen out of 26 (61.5%) GSD I patients in our study had suboptimal levels (<30 ng/ml) of 25-hydroxyvitamin-D (25(OH)D) despite supplementation of vitamin D and/or vitamin D + calcium based on WHO standards in 24/26 (92.3%) patients. The restrictive nature of the GSD I diet, metabolic derangements and intestinal malabsorption seen in GSD I are possible reasons for the observed hypovitaminosis D. Our results suggest that measurement of 25(OH)D should be considered in the routine evaluation of GSD I patients.
Full Text
Duke Authors
Cited Authors
- Banugaria, SG; Austin, SL; Boney, A; Weber, TJ; Kishnani, PS
Published Date
- April 2010
Published In
Volume / Issue
- 99 / 4
Start / End Page
- 434 - 437
PubMed ID
- 20060350
Electronic International Standard Serial Number (EISSN)
- 1096-7206
Digital Object Identifier (DOI)
- 10.1016/j.ymgme.2009.12.012
Language
- eng
Conference Location
- United States