| Dietary dilemmas in the management of glycogen storage disease type I. | |
| | |
MedLine Citation:
|
PMID: 21491105 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
|
Over the last 50 years, understanding the biochemical bases of glycogen storage disease type I has led to vastly improved survival and health outcomes but the management still centres around an extremely intensive dietary regimen. Patients' metabolic profiles are really determined by the whole of the diet and it can be very difficult to adjust therapy accordingly. In an iso-energetic diet with reference total energy intake, high carbohydrate intake could compromise other macro- and micro-nutrients; if carbohydrates are not restricted then total energy intake is excessive. The quality of the macronutrient such as the glycemic index of carbohydrate, the type of sugar and the proportion of medium-chain triglyceride and essential fatty acids also has a bearing on an individual's long-term metabolic control with potential clinical correlates. These factors as well as the different requirements between individuals and within individuals as they get older mean that the management of glycogen storage disease type I is particularly fraught. Regular clinical and dietary review is imperative as patients grow, ensuring adequate but not excessive low glycaemic index carbohydrate intake, appropriate dynamic biochemical profiles and suitable age appropriate eating patterns. Without diligent management, and education that empowers the patient, these individuals can struggle in adult life. |
| | |
Authors:
|
Kaustuv Bhattacharya |
Related Documents
:
|
20156465 - Physiological variables explain mineral intake in iberian red deer. 20105545 - Technical note: ruminal cannulation technique in young holstein calves: effects of cann... 18487665 - The effects of teat bar design and weaning method on behavior, intake, and gain of dair... 1596205 - The relationship between 48-h dental plaque accumulation in young human adults and the ... 4070415 - Influence of sympathectomy on body weight of rats given chow or supermarket diets. 16632175 - Influence of maternal dietary calcium levels on milk zinc, calcium and phosphorus conte... |
Publication Detail:
|
Type: JOURNAL ARTICLE Date: 2011-4-14 |
Journal Detail:
|
Title: Journal of inherited metabolic disease Volume: - ISSN: 1573-2665 ISO Abbreviation: - Publication Date: 2011 Apr |
Date Detail:
|
Created Date: 2011-4-14 Completed Date: - Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 7910918 Medline TA: J Inherit Metab Dis Country: - |
Other Details:
|
Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
|
Childrens' Hospital at Westmead, Westmead, NSW, Australia, Kaustuvb@chw.edu.au. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Clinical features of adult-onset chronic active Epstein-Barr virus infection: a retrospective analys...
Next Document: Tackling frontal lobe-related functions in PKU through functional brain imaging: a Stroop task in ad...