{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T02:49:44Z","timestamp":1780368584474,"version":"3.54.1"},"reference-count":22,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[1983,12,1]],"date-time":"1983-12-01T00:00:00Z","timestamp":439084800000},"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":[[1983,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We successfully treated a critically ill infant with the classical type of maple syrup urine disease by multiple exchange tranfusions via a peripheral artery and vein and with positive supplementation in the early stage of therapy. Clinical improvement was definite after the plasma leucine level fell below 1 mmol\/l. There was a close linear correlation between plasma concentrations of branched\u2010chain amino acids and their corresponding branched\u2010chain \u03b1\u2010keto acids and branched\u2010chain \u03b1\u2010hydroxy acids. \u03b1\u2010Hydroxy acids were more easily excreted in the urine than \u03b1\u2010keto acids and amino acids. Our studies on urinary organic acids supported the existence of minor metabolic pathways of branched\u2010chain \u03b1\u2010keto acids, although they were not though to be important in eliminating accumulated \u03b1\u2010keto acids. Urinary excretion of succinic acid and \u03b1\u2010ketoglutaric acid, which are components of the citric acid cycle, increased transiently during the patient's convalescence. The cerebrospinal fluid\/plasma ratios for branched\u2010chain amino acids, \u03b1\u2010keto acids, and \u03b1\u2010hydroxy acids were very high before the transfurions and decreased after improvement. The cerebrospinal fluid\/plasma rations for 5\u2010carvon acids, \u03b1\u2010ketoisovaleric acid and \u03b1\u2010hydroxyisiovaleric acid were much higher than for other branched\u2010chain acids not only in the patients but also in normal controls. Cerebrospinal fluid levels of \u03b1\u2010ketoisocaproic acid and \u03b1\u2010hydroxyisovaleric acid were as high as 1 mmol\/l in our patient.<\/jats:p>","DOI":"10.1007\/bf02310879","type":"journal-article","created":{"date-parts":[[2006,2,15]],"date-time":"2006-02-15T15:54:22Z","timestamp":1140018862000},"page":"183-189","source":"Crossref","is-referenced-by-count":15,"title":["Organic acids and branched\u2010chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease"],"prefix":"10.1002","volume":"6","author":[{"given":"Y.","family":"Shigematsu","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine Kyoto University  Sakyo\u2010ku Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Kikuchi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine Kyoto University  Sakyo\u2010ku Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Momoi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine Kyoto University  Sakyo\u2010ku Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Sudo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukui Medical School  Fukui Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y.","family":"Kikawa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukui Medical School  Fukui Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Nosaka","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukui Prefectural Hospital  Fukui Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Kuriyama","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukui Prefectural Hospital  Fukui Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Haruki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukui Prefectural Hospital  Fukui Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Sanada","sequence":"additional","affiliation":[{"name":"Division of Clinical Nutrition Kyoto University Hospital  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Hamano","sequence":"additional","affiliation":[{"name":"Division of Clinical Nutrition Kyoto University Hospital  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y.","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Chest Disease Research Institute Kyoto University  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1983,12]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1542\/peds.42.3.395","article-title":"Endogenous renal transport of free amino acids in infancy and childhood","volume":"42","author":"Brodehl J.","year":"1968","journal-title":"Pediatrics"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(79)80169-9"},{"key":"e_1_2_1_4_1","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1542\/peds.68.6.856","article-title":"Outcome of early and long\u2010term management of classical maple syrup urine disease","volume":"68","author":"Clow C. 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