{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T19:39:52Z","timestamp":1790969992353,"version":"4.1.0"},"reference-count":17,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["brainanddevelopment.com","clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Brain and Development"],"published-print":{"date-parts":[[2022,3]]},"DOI":"10.1016\/j.braindev.2021.11.005","type":"journal-article","created":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T18:20:26Z","timestamp":1638382826000},"page":"244-248","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":23,"title":["Intravenous ketogenic diet therapy for neonatal-onset pyruvate dehydrogenase complex deficiency"],"prefix":"10.1016","volume":"44","author":[{"given":"Takehiko","family":"Inui","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoichi","family":"Wada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moriei","family":"Shibuya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natsuko","family":"Arai-Ichinoi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukimune","family":"Okubo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wakaba","family":"Endo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshihiko","family":"Uchida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noriko","family":"Togashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Etsuo","family":"Naito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuhiro","family":"Haginoya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"11","key":"10.1016\/j.braindev.2021.11.005_b0005","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1136\/jmg.31.11.875","article-title":"Pyruvate dehydrogenase deficiency","volume":"31","author":"Brown","year":"1994","journal-title":"J Med Genet"},{"issue":"3","key":"10.1016\/j.braindev.2021.11.005_b0010","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1007\/s10545-014-9787-3","article-title":"The spectrum of pyruvate oxidation defects in the diagnosis of mitochondrial disorders","volume":"38","author":"Sperl","year":"2015","journal-title":"J Inherit Metab Dis"},{"issue":"3","key":"10.1016\/j.braindev.2021.11.005_b0015","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1016\/j.ymgme.2012.03.017","article-title":"The spectrum of pyruvate dehydrogenase complex deficiency: Clinical, biochemical and genetic features in 371 patients","volume":"106","author":"Patel","year":"2012","journal-title":"Mol Genet Metab"},{"key":"10.1016\/j.braindev.2021.11.005_b0020","doi-asserted-by":"crossref","unstructured":"Barnerias C, Saudubray JM, Touati G, De Lonlay P, Dulac O, Ponsot G, et al. Pyruvate dehydrogenase complex deficiency: Four neurological phenotypes with differing pathogenesis. Dev Med Child Neurol 2010;52:e1-9.","DOI":"10.1111\/j.1469-8749.2009.03541.x"},{"issue":"5","key":"10.1016\/j.braindev.2021.11.005_b0025","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1542\/peds.2007-3502","article-title":"Mitochondrial oxidative phosphorylation disorders presenting in neonates: Clinical manifestations and enzymatic and molecular diagnoses","volume":"122","author":"Gibson","year":"2008","journal-title":"Pediatrics"},{"issue":"2","key":"10.1016\/j.braindev.2021.11.005_b0030","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1007\/s10545-016-0011-5","article-title":"Ketogenic diet in pyruvate dehydrogenase complex deficiency: short- and long-term outcomes","volume":"40","author":"Sofou","year":"2017","journal-title":"J Inherit Metab Dis"},{"issue":"9","key":"10.1016\/j.braindev.2021.11.005_b0035","doi-asserted-by":"crossref","first-page":"1354","DOI":"10.1007\/s00247-016-3585-z","article-title":"Prenatal presentation of pyruvate dehydrogenase complex deficiency","volume":"46","author":"Natarajan","year":"2016","journal-title":"Pediatr Radiol"},{"issue":"3","key":"10.1016\/j.braindev.2021.11.005_b0040","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1177\/1093526619876448","article-title":"Distinguishing encephaloclastic lesions resulting from primary or secondary pyruvate dehydrogenase deficiency from other neonatal or infantile cavitary brain lesions","volume":"23","author":"Gilani","year":"2020","journal-title":"Pediatr Dev Pathol"},{"key":"10.1016\/j.braindev.2021.11.005_b0045","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1542\/peds.58.5.713","article-title":"Ketonic diet in the management of pyruvate dehydrogenase deficiency","volume":"58","author":"Falk","year":"1976","journal-title":"Pediatrics"},{"issue":"1","key":"10.1016\/j.braindev.2021.11.005_b0050","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/S0925-4439(02)00142-4","article-title":"Thiamine-responsive pyruvate dehydrogenase deficiency in two patients caused by a point mutation (F205L and L216F) within the thiamine pyrophosphate binding region","volume":"1588","author":"Naito","year":"2002","journal-title":"Biochim Biophys Acta - Mol Basis Dis"},{"issue":"7","key":"10.1016\/j.braindev.2021.11.005_b0055","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1002\/acn3.73","article-title":"Phenylbutyrate increases pyruvate dehydrogenase complex activity in cells harboring a variety of defects","volume":"1","author":"Ferriero","year":"2014","journal-title":"Ann Clin Transl Neurol"},{"issue":"6","key":"10.1016\/j.braindev.2021.11.005_b0060","doi-asserted-by":"crossref","first-page":"1655","DOI":"10.1212\/WNL.49.6.1655","article-title":"Outcome of pyruvate dehydrogenase deficiency treated with ketogenic diets: Studies in patients with identical mutations","volume":"49","author":"Wexler","year":"1997","journal-title":"Neurology"},{"issue":"1","key":"10.1016\/j.braindev.2021.11.005_b0065","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1111\/dmcn.14306","article-title":"Optimal clinical management of children receiving ketogenic parenteral nutrition: a clinical practice guide","volume":"62","author":"Louw","year":"2020","journal-title":"Dev Med Child Neurol"},{"issue":"3","key":"10.1016\/j.braindev.2021.11.005_b0070","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/j.ymgme.2021.04.007","article-title":"REVIEW: Practical strategies to maintain anabolism by intravenous nutritional management in children with inborn metabolic diseases","volume":"133","author":"Kripps","year":"2021","journal-title":"Mol Genet Metab"},{"issue":"7","key":"10.1016\/j.braindev.2021.11.005_b0075","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1016\/j.braindev.2016.01.002","article-title":"Outcome of ketogenic diets in GLUT1 deficiency syndrome in Japan: A nationwide survey","volume":"38","author":"Fujii","year":"2016","journal-title":"Brain Dev"},{"issue":"40","key":"10.1016\/j.braindev.2021.11.005_b0080","doi-asserted-by":"crossref","first-page":"6133","DOI":"10.3748\/wjg.14.6133","article-title":"Nutritional management of newborn infants: Practical guidelines","volume":"14","author":"Ben","year":"2008","journal-title":"World J Gastroenterol"},{"issue":"6","key":"10.1016\/j.braindev.2021.11.005_b0085","doi-asserted-by":"crossref","first-page":"2315","DOI":"10.1016\/j.clnu.2018.06.945","article-title":"ESPGHAN\/ESPEN\/ESPR\/CSPEN guidelines on pediatric parenteral nutrition: Amino acids","volume":"37","author":"van Goudoever","year":"2018","journal-title":"Clin Nutr"}],"container-title":["Brain and Development"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0387760421002102?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0387760421002102?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T19:50:16Z","timestamp":1759953016000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0387760421002102"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3]]},"references-count":17,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2022,3]]}},"alternative-id":["S0387760421002102"],"URL":"https:\/\/doi.org\/10.1016\/j.braindev.2021.11.005","relation":{},"ISSN":["0387-7604"],"issn-type":[{"value":"0387-7604","type":"print"}],"subject":[],"published":{"date-parts":[[2022,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Intravenous ketogenic diet therapy for neonatal-onset pyruvate dehydrogenase complex deficiency","name":"articletitle","label":"Article Title"},{"value":"Brain and Development","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.braindev.2021.11.005","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}