{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T08:43:06Z","timestamp":1762504986575},"reference-count":11,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2004,10,7]],"date-time":"2004-10-07T00:00:00Z","timestamp":1097107200000},"content-version":"vor","delay-in-days":8225,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1982,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A child with debrancher deficiency presented with myopathy, recurrent hypoglycemia, and growth failure. Evidence for enhanced gluconeogenesis was demonstrated by low postabsorptive gluconeogenic plasma amino acids, a marked fall in alanine during fasting, and a substantial rise in plasma glucose following protein ingestion. The patient was treated with high\u2010protein nocturnal intragastric therapy, which resulted in marked improvement in exercise tolerance, muscle strength and mass, electromyographic findings, and growth.<\/jats:p>","DOI":"10.1002\/ana.410110417","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T13:30:35Z","timestamp":1104586235000},"page":"420-422","source":"Crossref","is-referenced-by-count":46,"title":["Reversal of debrancher deficiency myopathy by the use of high\u2010protein nutrition"],"prefix":"10.1002","volume":"11","author":[{"given":"Alfred E.","family":"Slonim","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charlene","family":"Weisberg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Benke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Owen B.","family":"Evans","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian M.","family":"Burr","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,7]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1971.00490020089011"},{"key":"e_1_2_1_3_2","first-page":"331","article-title":"Glycogen storage disease: new approaches to therapy","volume":"55","author":"Crigler JF","year":"1978","journal-title":"Ciba Found Symp"},{"key":"e_1_2_1_4_2","first-page":"123","article-title":"Disorders of glycogen and lipid metabolism","volume":"17","author":"DiMauro S","year":"1977","journal-title":"Adv Neurol"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410050504"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.bi.44.070175.004441"},{"key":"e_1_2_1_7_2","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1542\/peds.41.5.935","article-title":"Hexose and protein tolerance tests in children with liver glycogenosis caused by a deficiency of the debranching enzyme system","volume":"41","author":"Fernandes J","year":"1968","journal-title":"Pediatrics"},{"key":"e_1_2_1_8_2","first-page":"2301","article-title":"Regulation and significance of amino acid metabolism in skeletal muscle","volume":"37","author":"Goldberg AL","year":"1978","journal-title":"Fed Proc"},{"key":"e_1_2_1_9_2","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/S0065-3101(22)00561-8","article-title":"Inborn errors of glycogen metabolism","volume":"19","author":"Moses SW","year":"1972","journal-title":"Adv Pediatr"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(73)90190-1"},{"key":"e_1_2_1_11_2","first-page":"243","article-title":"Myopathy associated with glycogen deposition resulting from generalized lack of amylo\u20101,6\u2010glucosidase","volume":"9","author":"Oliner L","year":"1961","journal-title":"Clin Res"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196810312791803"}],"container-title":["Annals of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fana.410110417","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ana.410110417","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,2]],"date-time":"2023-11-02T17:17:13Z","timestamp":1698945433000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ana.410110417"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1982,4]]},"references-count":11,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1982,4]]}},"alternative-id":["10.1002\/ana.410110417"],"URL":"https:\/\/doi.org\/10.1002\/ana.410110417","archive":["Portico"],"relation":{},"ISSN":["0364-5134","1531-8249"],"issn-type":[{"value":"0364-5134","type":"print"},{"value":"1531-8249","type":"electronic"}],"subject":[],"published":{"date-parts":[[1982,4]]}}}