{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,30]],"date-time":"2026-05-30T00:26:19Z","timestamp":1780100779135,"version":"3.54.0"},"reference-count":6,"publisher":"Wiley","issue":"S1","license":[{"start":{"date-parts":[[1984,3,1]],"date-time":"1984-03-01T00:00:00Z","timestamp":446947200000},"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":[[1984,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Various types of dicarboxylic aciduria are known, most of them are accompanied by non\u2010ketotic hypoglycaemia. For the differential diagnosis of these conditions several methods of investigation have been used: (1) analysis of urinary organic acids in both native and hydrolysed samples, (2) analysis of free and esterified carnitine, the latter by means of chromatographic separation and identification of acyl moieties, (3) analysis of plasma organic acids, including the so\u2010called free fatty acids, (4) a prolonged fasting test with serial measurements of the aforementioned parameters and close monitoring of the blood glucose and (5) an oral loading test with medium chain triglycerides accompanied by the same measurements as those named in item (4).<\/jats:p><jats:p>So far differentiation has been made between patients with a metabolite profile most probably characteristic of medium chain acyl\u2010CoA dehydrogenase deficiency and other dicarboxylic acidurias, among the latter systemic carnitine deficiency. Patients belonging to the first group accumulate octanoate, decanoate and<jats:italic>cis<\/jats:italic>\u20104\u2010decenoate in their plasma; they excrete hexanoylglycine, octanoylcarnitine and suberylglycine in addition to the usual C<jats:sub>6<\/jats:sub>\u2013C<jats:sub>10<\/jats:sub> dicarboxylic acids. There was a high prevalence of an increased plasma free fatty acid\/3\u2010hydroxybutyrate ratio.<\/jats:p>","DOI":"10.1007\/bf03047374","type":"journal-article","created":{"date-parts":[[2009,6,25]],"date-time":"2009-06-25T03:36:15Z","timestamp":1245900975000},"page":"48-51","source":"Crossref","is-referenced-by-count":34,"title":["The differential diagnosis of dicarboxylic aciduria"],"prefix":"10.1002","volume":"7","author":[{"given":"M.","family":"Duran","sequence":"first","affiliation":[{"name":"University Children's Hospital \u2018Het Wilhelmina Kinder ziekenhuis\u2019  Nieuwe Gracht 137 Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. B. C.","family":"De Klerk","sequence":"additional","affiliation":[{"name":"University Children's Hospital \u2018Het Wilhelmina Kinder ziekenhuis\u2019  Nieuwe Gracht 137 Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. K.","family":"Wadman","sequence":"additional","affiliation":[{"name":"University Children's Hospital \u2018Het Wilhelmina Kinder ziekenhuis\u2019  Nieuwe Gracht 137 Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Bruinvis","sequence":"additional","affiliation":[{"name":"University Children's Hospital \u2018Het Wilhelmina Kinder ziekenhuis\u2019  Nieuwe Gracht 137 Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Ketting","sequence":"additional","affiliation":[{"name":"University Children's Hospital \u2018Het Wilhelmina Kinder ziekenhuis\u2019  Nieuwe Gracht 137 Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1984,3]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(83)90186-9"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01810357"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(76)90355-7"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198210000-00012"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.25.1.16"},{"key":"e_1_2_1_7_1","first-page":"1758","article-title":"Carnitine and acylcarnitines","volume":"14","author":"Pearson D. J.","year":"1969","journal-title":"Meth. Enzymol."}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF03047374","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T00:32:54Z","timestamp":1694824374000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/BF03047374"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1984,3]]},"references-count":6,"journal-issue":{"issue":"S1","published-print":{"date-parts":[[1984,3]]}},"alternative-id":["10.1007\/BF03047374"],"URL":"https:\/\/doi.org\/10.1007\/bf03047374","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1984,3]]}}}