{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,18]],"date-time":"2025-10-18T10:37:29Z","timestamp":1760783849374},"reference-count":40,"publisher":"Walter de Gruyter GmbH","issue":"1-2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>To determine the individual fasting tolerance for patients with glycogen storage disease type III (GSD III) and to assess their linear growth velocity after tailoring of dose intervals of oral uncooked cornstarch.<\/jats:p><jats:p>A prospective cohort study included 32 patients with GSD III aged 6 months\u201311.5 years (median: 3.3 years). The fasting tolerance of each patient was determined as the time interval between starch administration until the drop in blood glucose level was below 60 mg\/dL.<\/jats:p><jats:p>Some 27 patients (84.4%) developed hypoglycemia. The intervals between oral cornstarch administration were tailored for each child according to his\/her individual fasting tolerance. After a 6-month follow up there was a significant reduction in seizure attacks (p&lt;0.01) and liver size (p&lt;0.01), but there was no statistically significant difference in liver transaminase and serum lactate levels. There was a significant improvement in height (p&lt;0.01) and linear growth velocity (p&lt;0.05) of these patients after at least a 12-month follow up.<\/jats:p><jats:p>: Adjusting the intervals between the cornstarch doses for each patient with GSD III, according to individual fasting tolerance test was very beneficial and resulted in improvement of the linear growth velocity and reduction in the frequency of hypoglycemic seizures as well as the size of the liver. Individual scheduling of cornstarch doses prevents complications in those who develop hypoglycemia at short intervals; it also allows some relaxation in schedule for those who can tolerate longer fasting hours to improve their appetite and prolong their uninterrupted sleep hours.<\/jats:p>","DOI":"10.1515\/jpem-2014-0145","type":"journal-article","created":{"date-parts":[[2014,8,20]],"date-time":"2014-08-20T17:02:52Z","timestamp":1408554172000},"source":"Crossref","is-referenced-by-count":7,"title":["The effect of tailoring of cornstarch intake on stature in children with glycogen storage disease type III"],"prefix":"10.1515","volume":"28","author":[{"given":"Hanaa","family":"El-Karaksy","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mona S.","family":"El-Raziky","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ghada","family":"Anwar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Engy","family":"Mogahed","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","reference":[{"key":"ref111","first-page":"63","article-title":"Psychosocial care of the child and family In van den editors Inborn metabolic diseases rd ed","author":"Harris","year":"2000"},{"key":"ref201","first-page":"658","article-title":"Lactate a preferred for human metabolism in vivo Flow","author":"Smith","year":"2003","journal-title":"fuel brain Blood"},{"key":"ref261","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1016\/S0957-5839(97)80191-1","article-title":"storage diseases","volume":"13","author":"Lee","year":"1997","journal-title":"Curr Paediatr"},{"key":"ref301","first-page":"95","article-title":"Wolfsdorf DA storage diseases","author":"Weinstein","year":"2003","journal-title":"Rev"},{"key":"ref271","first-page":"1993","article-title":"DA Glycogen storage disease type III In editors Internet University of Accessed updated September","author":"Dagli","year":"2014","journal-title":"Seattle Seattle"},{"key":"ref321","first-page":"1","article-title":"DA Wolfsdorf Effect of continuous glucose therapy with uncooked cornstarch on the long - term clinical course of type a glycogen storage disease","volume":"35","author":"Weinstein","year":"2002","journal-title":"Eur J Pediatr"},{"key":"ref191","first-page":"161","article-title":"Guidelines for management of glycogen storage disease type European study on glycogen storage disease type","author":"Rake","year":"2002","journal-title":"Eur J Pediatr"},{"key":"ref161","article-title":"Glycogen storage diseases In editors The metabolic and molecular bases of inherited disease New York","author":"Chen","year":"2001"},{"key":"ref181","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1007\/s10545-008-0919-5","article-title":"Hyperlipidemia in glycogen storage disease type III : effect of age and metabolic control","volume":"31","author":"Bernier","year":"2008","journal-title":"J Inherit Metab Dis"},{"key":"ref241","article-title":"therapy for hepatic glycogen storage diseases","volume":"30","author":"Goldberg","year":"1993","journal-title":"Nutrition J Am Diet Assoc"},{"key":"ref81","first-page":"95","article-title":"Wolfsdorf DA storage diseases","author":"Weinstein","year":"2003","journal-title":"Rev"},{"key":"ref281","first-page":"238","article-title":"Glycogen storage disease : a basic understanding and guide to nursing care","volume":"12","author":"Triomphe","year":"1997","journal-title":"J Pediatr"},{"key":"ref231","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1097\/MPG.0b013e3181818ea5","article-title":"Nutritional therapy for glycogen storage diseases","author":"Heller","year":"2008","journal-title":"J Pediatr Gastroenterol"},{"key":"ref101","first-page":"1","article-title":"DA Wolfsdorf Effect of continuous glucose therapy with uncooked cornstarch on the long - 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