{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:53:48Z","timestamp":1790805228820,"version":"4.1.0"},"reference-count":22,"publisher":"Wiley","issue":"S1","license":[{"start":{"date-parts":[[2009,3,30]],"date-time":"2009-03-30T00:00:00Z","timestamp":1238371200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2009,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Glycogen storage disease type III (GSD III) is caused by a deficiency in debranching enzyme, which leads to an accumulation of abnormal glycogen called limit dextrin in affected tissues. Muscle and liver involvement is present in GSD type IIIa, while the defect is limited to the liver only in GSD type IIIb. Besides skeletal muscle involvement, a cardiomyopathy resembling idiopathic hypertrophic cardiomyopathy is seen. Management consists of maintaining normoglycaemia by supplementation with cornstarch therapy and\/or protein. While studies are lacking regarding the best treatment for skeletal muscle disease, a high\u2010protein diet was previously reported to be beneficial. No cases of improvement in cardiomyopathy have been reported. Our patient presented in infancy with hypoglycaemia and hepatomegaly. His prescribed management consisted of cornstarch supplementation and a high\u2010protein diet providing 20% of his total energy needs. At 16 years of age, he developed a severe cardiomyopathy with a left ventricular mass index of 209 g\/m<jats:sup>2<\/jats:sup>. The cardiomyopathy remained stable on a protein intake of 20\u201325% of total energy. At age 22 years, the diet was changed to increase his protein intake to 30% of total energy and minimize his cornstarch therapy to only what was required to maintain normoglycaemia. Dramatic improvement in the cardiomyopathy occurred. Over one year, his left ventricular mass index decreased from 159.7 g\/m<jats:sup>2<\/jats:sup> to 78 g\/m<jats:sup>2<\/jats:sup> (normal 50\u201386 g\/m<jats:sup>2<\/jats:sup>) and the creatine kinase levels decreased from 455 U\/L to 282 U\/L. Avoidance of overtreatment with carbohydrate and a high\u2010protein diet can reverse and may prevent cardiomyopathy.<\/jats:p>","DOI":"10.1007\/s10545-009-1088-x","type":"journal-article","created":{"date-parts":[[2009,3,25]],"date-time":"2009-03-25T16:37:10Z","timestamp":1237999030000},"page":"103-106","source":"Crossref","is-referenced-by-count":65,"title":["Reversal of glycogen storage disease type IIIa\u2010related cardiomyopathy with modification of diet"],"prefix":"10.1002","volume":"32","author":[{"given":"A. I.","family":"Dagli","sequence":"first","affiliation":[{"name":"Raymond C. Philip Research and Education Unit, Division of Genetics, Department of Pediatrics University of Florida Gainesville Florida USA"},{"name":"Division of Pediatric Endocrinology and Glycogen Storage Disease Program, Department of Pediatrics University of Florida Gainesville Florida USA"},{"name":"Glycogen Storage Disease Program University of Florida College of Medicine Box 100296 Gainesville FL 32610\u20100296 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. T.","family":"Zori","sequence":"additional","affiliation":[{"name":"Raymond C. Philip Research and Education Unit, Division of Genetics, Department of Pediatrics University of Florida Gainesville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"McCune","sequence":"additional","affiliation":[{"name":"Raymond C. Philip Research and Education Unit, Division of Genetics, Department of Pediatrics University of Florida Gainesville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Ivsic","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Pediatrics University of Florida Gainesville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. K.","family":"Maisenbacher","sequence":"additional","affiliation":[{"name":"Raymond C. Philip Research and Education Unit, Division of Genetics, Department of Pediatrics University of Florida Gainesville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. A.","family":"Weinstein","sequence":"additional","affiliation":[{"name":"Division of Pediatric Endocrinology and Glycogen Storage Disease Program, Department of Pediatrics University of Florida Gainesville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,3,30]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.eurheartj.a015283"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-1119(97)00291-6"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(75)80254-7"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01744800"},{"key":"e_1_2_4_6_1","first-page":"1521","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Chen YT","year":"2001"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-116-11-896"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410050504"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.36.4.431"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(99)00038-3"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02238523"},{"key":"e_1_2_4_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9149(96)00885-5"},{"key":"e_1_2_4_13_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00219a036"},{"key":"e_1_2_4_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.34.8.862"},{"key":"e_1_2_4_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320420514"},{"key":"e_1_2_4_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00443106"},{"key":"e_1_2_4_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9149(84)90551-4"},{"key":"e_1_2_4_18_1","first-page":"303","article-title":"Glycogenosis type III myocardiopathy","volume":"36","author":"Rossignol AM","year":"1979","journal-title":"Arch Fr Pediatr"},{"key":"e_1_2_4_19_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI118799"},{"key":"e_1_2_4_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410110417"},{"key":"e_1_2_4_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(84)80075-X"},{"key":"e_1_2_4_22_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-120-3-199402010-00008"},{"key":"e_1_2_4_23_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1021831621210"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-009-1088-x","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1007\/s10545-009-1088-x","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-009-1088-x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T03:19:16Z","timestamp":1694747956000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-009-1088-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,3,30]]},"references-count":22,"journal-issue":{"issue":"S1","published-print":{"date-parts":[[2009,12]]}},"alternative-id":["10.1007\/s10545-009-1088-x"],"URL":"https:\/\/doi.org\/10.1007\/s10545-009-1088-x","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,3,30]]}}}