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Dietary dilemmas in the management of glycogen storage disease type I
Author(s) -
Bhattacharya Kaustuv
Publication year - 2011
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-011-9322-8
Subject(s) - dietary management , glycogen , medicine , glycogen storage disease , disease , type 2 diabetes , glycogen storage disease type i , glycemic , glycemic load , glycemic index , diabetes mellitus , intensive care medicine , biology , physiology , endocrinology
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.