{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T11:35:47Z","timestamp":1793878547828,"version":"4.7.0"},"reference-count":24,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2014,11,28]],"date-time":"2014-11-28T00:00:00Z","timestamp":1417132800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Orphanet J Rare Dis"],"published-print":{"date-parts":[[2014,12]]},"DOI":"10.1186\/s13023-014-0196-3","type":"journal-article","created":{"date-parts":[[2014,11,28]],"date-time":"2014-11-28T14:31:40Z","timestamp":1417185100000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":60,"title":["Glycogen storage disease type III: modified Atkins diet improves myopathy"],"prefix":"10.1186","volume":"9","author":[{"given":"Sebene","family":"Mayorandan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uta","family":"Meyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans","family":"Hartmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anibh Martin","family":"Das","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2014,11,28]]},"reference":[{"key":"196_CR1","doi-asserted-by":"crossref","unstructured":"Das AM, Steuerwald U, Illsinger S (2010): Inborn Errors of Energy Metabolism Associated with Myopathies. J Biomed Biotechnol 2010. http:\/\/dx.doi.org\/10.1155\/2010\/340849.,","DOI":"10.1155\/2010\/340849"},{"issue":"5","key":"196_CR2","doi-asserted-by":"publisher","first-page":"422","DOI":"10.1002\/ana.410050504","volume":"5","author":"S Dimauro","year":"1979","unstructured":"Dimauro S, Hartwig GB, Hays A, Eastwood AB, Franco R, Olarte M, Chang M, Roses AD, Fetell M, Schoenfeldt RS, Stern LZ: Debrancher deficiency: neuromuscular disorder in 5 adults. Ann Neurol. 1979, 5 (5): 422-436. 10.1002\/ana.410050504.","journal-title":"Ann Neurol"},{"issue":"8","key":"196_CR3","doi-asserted-by":"publisher","first-page":"862","DOI":"10.1136\/hrt.34.8.862","volume":"34","author":"CG Miller","year":"1972","unstructured":"Miller CG, Alleyne GA, Brooks SE: Gross cardiac involvement in glycogen storage disease type III. Br Heart J. 1972, 34 (8): 862-864. 10.1136\/hrt.34.8.862.","journal-title":"Br Heart J"},{"issue":"7","key":"196_CR4","doi-asserted-by":"publisher","first-page":"980","DOI":"10.1016\/0002-9149(84)90551-4","volume":"53","author":"LJ Olson","year":"1984","unstructured":"Olson LJ, Reeder GS, Noller KL, Edwards WD, Howell RR, Michels VV: Cardiac involvement in glycogen storage disease III: morphologic and biochemical characterization with endomyocardial biopsy. Am J Cardiol. 1984, 53 (7): 980-981. 10.1016\/0002-9149(84)90551-4.","journal-title":"Am J Cardiol"},{"issue":"3","key":"196_CR5","doi-asserted-by":"publisher","first-page":"532","DOI":"10.1093\/oxfordjournals.eurheartj.a015283","volume":"18","author":"H Akazawa","year":"1997","unstructured":"Akazawa H, Kuroda T, Kim S, Mito H, Kojo T, Shimada K: Specific heart muscle disease associated with glycogen storage disease type III: clinical similarity to the dilated phase of hypertrophic cardiomyopathy. Eur Heart J. 1997, 18 (3): 532-533. 10.1093\/oxfordjournals.eurheartj.a015283.","journal-title":"Eur Heart J"},{"issue":"4","key":"196_CR6","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1093\/ajcn\/52.4.671","volume":"52","author":"DA Gremse","year":"1990","unstructured":"Gremse DA, Bucuvalas JC, Balistrerie WF: Efficacy of cornstarch therapy in type III glycogen-storage disease. Am J Clin Nutr. 1990, 52 (4): 671-674.","journal-title":"Am J Clin Nutr"},{"issue":"3","key":"196_CR7","doi-asserted-by":"publisher","first-page":"226","DOI":"10.1007\/BF00442683","volume":"147","author":"J Fernandes","year":"1988","unstructured":"Fernandes J, Leonard JV, Moses SW, Odi\u00e8vre M, di Rocco M, Schaub J, Smit GP, Ullrich K, Durand P: Glycogen storage disease: recommendations for treatment. Eur J Pediatr. 1988, 147 (3): 226-228. 10.1007\/BF00442683.","journal-title":"Eur J Pediatr"},{"issue":"1","key":"196_CR8","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1023\/A:1021831621210","volume":"4","author":"JI Wolfsdorf","year":"2003","unstructured":"Wolfsdorf JI, Weinstein DA: Glycogen storage diseases. Rev Endocr Metab Disord. 2003, 4 (1): 95-102. 10.1023\/A:1021831621210.","journal-title":"Rev Endocr Metab Disord"},{"issue":"6","key":"196_CR9","doi-asserted-by":"publisher","first-page":"638","DOI":"10.1203\/PDR.0b013e318232154f","volume":"70","author":"V Valayannopoulos","year":"2011","unstructured":"Valayannopoulos V, Bajolle F, Arnoux JB, Dubois S, Sannier N, Baussan C, Petit F, Labrune P, Rabier D, Ottolenghi C, Vassault A, Broissand C, Bonnet D, De Lonlay P: Successful treatment of severe cardiomyopathy in glycogen storage disease type III with D, L -3-hydroxybutyrate, ketogenic and high-protein diet. Pediatr Res. 2011, 70 (6): 638-641. 10.1203\/PDR.0b013e318232154f.","journal-title":"Pediatr Res"},{"key":"196_CR10","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1007\/8904_2011_111","volume":"5","author":"CP Sentner","year":"2012","unstructured":"Sentner CP, Caliskan K, Vletter WB, Smit GPA: Heart failure due to severe hypertrophic cardiomyopathy reversed by low calorie, high protein dietary adjustments in a glycogen storage disease type IIIa patient. JIMD Rep. 2012, 5: 13-16. 10.1007\/8904_2011_111.","journal-title":"JIMD Rep"},{"issue":"Suppl 1","key":"196_CR11","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1007\/s10545-009-1088-x","volume":"32","author":"AI Dagli","year":"2009","unstructured":"Dagli AI, Zori RT, McCune H, Ivsic T, Maisenbacher MK, Weinstein DA: Reversal of glycogen storage disease type IIIa \u2013related cardiomyopathy with modification of diet. J Inherit Metab Dis. 2009, 32 (Suppl 1): 103-106. 10.1007\/s10545-009-1088-x.","journal-title":"J Inherit Metab Dis"},{"issue":"6\u20137","key":"196_CR12","doi-asserted-by":"publisher","first-page":"408","DOI":"10.1016\/S0960-8966(99)00038-3","volume":"9","author":"S Kiechl","year":"1999","unstructured":"Kiechl S, Willeit J, Vogel W, Kohlendorfer U, Poewe W: Reversible of severe myopathy of respiratory muscles due to adult-onset type III glycogenosis. Neuromuscul Disord. 1999, 9 (6\u20137): 408-410. 10.1016\/S0960-8966(99)00038-3.","journal-title":"Neuromuscul Disord"},{"issue":"2","key":"196_CR13","doi-asserted-by":"publisher","first-page":"145","DOI":"10.1016\/j.ymgme.2012.12.002","volume":"108","author":"B Sun","year":"2013","unstructured":"Sun B, Fredrickson K, Austin S, Tolun AA, Thurberg BL, Kraus WE, Bali D, Chen YT, Kishnani PS: Alglucosidase alfa enzyme replacement therapy as a therapeutic approach for glycogen storage disease type III. Mol Genet Metab. 2013, 108 (2): 145-147. 10.1016\/j.ymgme.2012.12.002.","journal-title":"Mol Genet Metab"},{"issue":"7","key":"196_CR14","doi-asserted-by":"publisher","first-page":"446","DOI":"10.1097\/GIM.0b013e3181e655b6","volume":"12","author":"PS Kishnani","year":"2010","unstructured":"Kishnani PS, Austin SL, Arn P, Bali DS, Boney A, Case LE, Chung WK, Desau DM, El-Gharbawy A, Haller R, Smit GPA, Smith AD, Hobson-Webb LD, Wechsler SB, Weinstein DA, Watson MS: Glygogen storage disease type III: diagnosis and management guidelines. Genet Med. 2010, 12 (7): 446-463. 10.1097\/GIM.0b013e3181e655b6.","journal-title":"Genet Med"},{"issue":"1","key":"196_CR15","first-page":"13","volume":"46","author":"H Vestergaard","year":"1999","unstructured":"Vestergaard H: Studies of gene expression and activity of hexokinase, phosphofructokinase and glycogen synthase in human skeletal muscle in states of altered insulin- stimulated glucose metabolism. Dan Med Bull. 1999, 46 (1): 13-34.","journal-title":"Dan Med Bull"},{"issue":"3","key":"196_CR16","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1016\/0033-0620(72)90029-1","volume":"15","author":"JR Neely","year":"1972","unstructured":"Neely JR, Rovetto MJ, Oram JF: Myocardial utilization of carbohydrate and lipids. Prog Cardiovasc Dis. 1972, 15 (3): 289-329. 10.1016\/0033-0620(72)90029-1.","journal-title":"Prog Cardiovasc Dis"},{"issue":"6","key":"196_CR17","doi-asserted-by":"publisher","first-page":"906","DOI":"10.1016\/S0022-3476(84)80075-X","volume":"105","author":"AE Slonim","year":"1984","unstructured":"Slonim AE, Coleman RA, Moses WS: Myopathy and growth failure in debrancher enzyme deficiency: improvement with high-protein nocturnal enteral therapy. J Pediatr. 1984, 105 (6): 906-911. 10.1016\/S0022-3476(84)80075-X.","journal-title":"J Pediatr"},{"key":"196_CR18","doi-asserted-by":"publisher","first-page":"65","DOI":"10.1007\/8904_2011_118","volume":"6","author":"SL Austin","year":"2012","unstructured":"Austin SL, Proia AD, Spencer-Manzon MJ, Butany J, Wechsler SB, Krishnani PS: Cardiac pathology in glycogen storage disease type III. JIMD Rep. 2012, 6: 65-72. 10.1007\/8904_2011_118.","journal-title":"JIMD Rep"},{"issue":"4","key":"196_CR19","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1007\/BF01799498","volume":"13","author":"GPA Smit","year":"1990","unstructured":"Smit GPA, Fernandes J, Leonard JV, Matthews EE, Moses SW, Odievre M, Ullrich K: The long-term outcome of patients with glycogen storage diseases. J Inherit Metab Dis. 1990, 13 (4): 411-418. 10.1007\/BF01799498.","journal-title":"J Inherit Metab Dis"},{"issue":"4","key":"196_CR20","doi-asserted-by":"publisher","first-page":"911","DOI":"10.1016\/0002-8703(95)90097-7","volume":"130","author":"H Tada","year":"1995","unstructured":"Tada H, Kurita T, Ohe T, Shimomura K, Ishihara T, Yamada Y, Osawa N: Glycogen storage disease type III associated with ventricular tachycardia. Am Heart J. 1995, 130 (4): 911-912. 10.1016\/0002-8703(95)90097-7.","journal-title":"Am Heart J"},{"issue":"1","key":"196_CR21","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1016\/j.ymgme.2013.02.008","volume":"109","author":"N Preisler","year":"2013","unstructured":"Preisler N, Pradel A, Husu E, Madsen KL, Becquemin MH, Mollet A, Labrune P, Petit F, Hogrel JY, Jardel C, Maillot F, Vissing J, Laforet P: Exercise intolerance in glycogen storage disease type III: weakness or energy deficiency?. Mol Genet Metab. 2013, 109 (1): 14-20. 10.1016\/j.ymgme.2013.02.008.","journal-title":"Mol Genet Metab"},{"issue":"Suppl 3","key":"196_CR22","doi-asserted-by":"publisher","first-page":"168","DOI":"10.1016\/j.nmd.2012.10.011","volume":"22","author":"J Vissing","year":"2012","unstructured":"Vissing J, Haller JG: Mechanisms of exertional fatigue in muscle glycogenoses. Neuromuscul Disord. 2012, 22 (Suppl 3): 168-171. 10.1016\/j.nmd.2012.10.011.","journal-title":"Neuromuscul Disord"},{"key":"196_CR23","doi-asserted-by":"publisher","first-page":"377","DOI":"10.1111\/j.1749-6632.2009.04704.x","volume":"1171","author":"M Balietti","year":"2009","unstructured":"Balietti M, Fattoretti P, Giorgetti B, Casoli T, Di Stefano G, Solazzi M, Platano D, Aicardi G, Bertoni- Freddari C: A ketogenic diet increases succinic dehydrogenase activity in aging cardiomyocytes. Ann N Y Acad Sci. 2009, 1171: 377-384. 10.1111\/j.1749-6632.2009.04704.x.","journal-title":"Ann N Y Acad Sci"},{"key":"196_CR24","doi-asserted-by":"publisher","first-page":"237","DOI":"10.1016\/j.seizure.2012.02.005","volume":"21","author":"S Auvin","year":"2012","unstructured":"Auvin S: Should we routinely use modified Atkins diet instead of regular ketogenic diet to treat children with epilepsy?. Seizure. 2012, 21: 237-240. 10.1016\/j.seizure.2012.02.005.","journal-title":"Seizure"}],"container-title":["Orphanet Journal of Rare Diseases"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-014-0196-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s13023-014-0196-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-014-0196-3","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-014-0196-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,2]],"date-time":"2021-09-02T06:31:49Z","timestamp":1630564309000},"score":1,"resource":{"primary":{"URL":"https:\/\/ojrd.biomedcentral.com\/articles\/10.1186\/s13023-014-0196-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,11,28]]},"references-count":24,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2014,12]]}},"alternative-id":["196"],"URL":"https:\/\/doi.org\/10.1186\/s13023-014-0196-3","relation":{},"ISSN":["1750-1172"],"issn-type":[{"value":"1750-1172","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,11,28]]},"assertion":[{"value":"31 July 2014","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 November 2014","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 November 2014","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"196"}}