{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T16:37:39Z","timestamp":1759941459821},"reference-count":11,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1993,3,1]],"date-time":"1993-03-01T00:00:00Z","timestamp":730944000000},"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":[[1993,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Two sisters with fructose\u20101,6\u2010diphosphatase deficiency are reported. They presented with ketonuria, elevated plasma transaminase activity and severe metabolic acidosis during hypoglycaemic crises, which resembled Reye syndrome. Intravenous fructose tolerance tests provoked severe hypoglycaemia and metabolic acidosis. Fructose\u20101,6\u2010diphosphatase activities in both peripheral leukocytes and cultured lymphocytes were below the limit of detection. Urinary organic acid analysis during crises revealed markedly increased excretion of lactate, ketone bodies, glycerol and glycerol\u20103\u2010phosphate. We newly identified other glycolytic intermediates, glyceraldehyde, 3\u2010phosphoglycerate and fructose\u20101,6\u2010diphosphate, in the urine during hypoglycaemic attacks or after fructose tolerance tests. Identification of such compounds may be useful in the early diagnosis of this disease.<\/jats:p>","DOI":"10.1007\/bf00710290","type":"journal-article","created":{"date-parts":[[2004,12,1]],"date-time":"2004-12-01T12:15:16Z","timestamp":1101903316000},"page":"408-414","source":"Crossref","is-referenced-by-count":14,"title":["Urinary sugar phosphates and related organic acids in fructose\u20101,6\u2010diphosphatase deficiency"],"prefix":"10.1002","volume":"16","author":[{"given":"A.","family":"Nakai","sequence":"first","affiliation":[{"name":"Department of Pediatrics Fukui Medical School  23 Shimoaizuki, Matsuoka Fukui 910\u201011 Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y.","family":"Shigematsu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukui Medical School  23 Shimoaizuki, Matsuoka Fukui 910\u201011 Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y. Y.","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukui Medical School  23 Shimoaizuki, Matsuoka Fukui 910\u201011 Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y.","family":"Kikawa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukui Medical School  23 Shimoaizuki, Matsuoka Fukui 910\u201011 Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Sudo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukui Medical School  23 Shimoaizuki, Matsuoka Fukui 910\u201011 Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1993,3]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(70)92474-8"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00451915"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(69)90311-X"},{"key":"e_1_2_1_5_1","first-page":"399","volume-title":"The Metabolic Basis of Inherited Disease","author":"Gitzelmann R","year":"1989"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(84)90163-3"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01800490"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0076-6879(82)89004-6"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(73)80591-8"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199107000-00002"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI107018"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(78)90593-4"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF00710290","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF00710290","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T16:31:06Z","timestamp":1694795466000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/BF00710290"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,3]]},"references-count":11,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1993,3]]}},"alternative-id":["10.1007\/BF00710290"],"URL":"https:\/\/doi.org\/10.1007\/bf00710290","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,3]]}}}