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Patients\u2019 metabolic profiles are really determined by the whole of the diet and it can be very difficult to adjust therapy accordingly. In an iso\u2010energetic diet with reference total energy intake, high carbohydrate intake could compromise other macro\u2010 and micro\u2010nutrients; 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\u2013chain triglyceride and essential fatty acids also has a bearing on an individual's long\u2010term 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.<\/jats:p>","DOI":"10.1007\/s10545-011-9322-8","type":"journal-article","created":{"date-parts":[[2011,4,13]],"date-time":"2011-04-13T06:22:26Z","timestamp":1302675746000},"page":"621-629","source":"Crossref","is-referenced-by-count":40,"title":["Dietary dilemmas in the management of glycogen storage disease type I"],"prefix":"10.1002","volume":"34","author":[{"given":"Kaustuv","family":"Bhattacharya","sequence":"first","affiliation":[{"name":"Childrens\u2019 Hospital at Westmead Westmead NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,4,14]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1989.133"},{"key":"e_1_2_9_3_1","doi-asserted-by":"crossref","unstructured":"BandsmaRH SmitGP ReijngoudDJ KuipersF(2009)Adiponectin levels correlate with the severity of hypertriglyceridaemia in glycogen storage disease Ia.J Inherit Metab Dis.","DOI":"10.1007\/s10545-009-0993-3"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2009.12.012"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2008.10.048"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-007-0479-0"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.2337\/diab.26.11.1016"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/00042737-200211000-00014"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02072043"},{"key":"e_1_2_9_10_1","first-page":"1521","volume-title":"The metabolic and molecular bases of inherited disease","author":"Chen YT","year":"2001"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198401193100306"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02072090"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799686"},{"key":"e_1_2_9_14_1","doi-asserted-by":"crossref","unstructured":"CorreiaC BhattacharyaK ShusterJJet al. 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