{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,20]],"date-time":"2025-09-20T21:27:48Z","timestamp":1758403668549},"reference-count":10,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1983,10,31]],"date-time":"1983-10-31T00:00:00Z","timestamp":436406400000},"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":[[1984,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A new patient with medium\u2010chain dicarboxylic aciduria and suberyl glycinuria during an attack of acute illness is reported. When, inadvertently he was given medium\u2010chain triglycerides for 2 days, the excretion of abnormal metabolites of medium\u2010chain fatty acids increased and hepatomegaly became more pronounced. During remission a low excretion of the metabolites were observed. After 16 h of fasting hypoglycaemia was accompanied by an increase of urinary dicarboxylic acids and \u03c8\u2010hydroxyacids similar to that found on admission. Interestingly this urinary organic acid pattern persisted 8 h after intravenous administration of glucose. In a blood sample obtained after 16 h of fasting there was hypoketonaemia and increased levels of total free fatty acids, octanoic, decanoic and<jats:italic>cis<\/jats:italic>\u20104\u2010decenoic acids. These biochemical data suggest the existence of a deficiency at the level of medium\u2010chain acyl\u2010CoA dehydrogenase.<\/jats:p>","DOI":"10.1007\/bf01805804","type":"journal-article","created":{"date-parts":[[2005,6,27]],"date-time":"2005-06-27T23:18:31Z","timestamp":1119914311000},"page":"62-64","source":"Crossref","is-referenced-by-count":20,"title":["A new patient with dicarboxylic aciduria suggestive of medium\u2010chain acyl\u2010CoA dehydrogenase deficiency presenting as Reye's syndrome"],"prefix":"10.1002","volume":"7","author":[{"given":"J. A.","family":"Del Valle","sequence":"first","affiliation":[{"name":"Centro de Diagn\u00f3stico de Enfermedades Moleculares, Dpto. de Bioquimica y Biolog\u00eda Molecular, Facultad de Ciencias Universidad Aut\u00f3noma  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. J.","family":"Garcia","sequence":"additional","affiliation":[{"name":"Centro de Diagn\u00f3stico de Enfermedades Moleculares, Dpto. de Bioquimica y Biolog\u00eda Molecular, Facultad de Ciencias Universidad Aut\u00f3noma  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Merinero","sequence":"additional","affiliation":[{"name":"Centro de Diagn\u00f3stico de Enfermedades Moleculares, Dpto. de Bioquimica y Biolog\u00eda Molecular, Facultad de Ciencias Universidad Aut\u00f3noma  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"P\u00e9rez\u2010Cerd\u00e1","sequence":"additional","affiliation":[{"name":"Centro de Diagn\u00f3stico de Enfermedades Moleculares, Dpto. de Bioquimica y Biolog\u00eda Molecular, Facultad de Ciencias Universidad Aut\u00f3noma  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F.","family":"Roman","sequence":"additional","affiliation":[{"name":"Centro de Diagn\u00f3stico de Enfermedades Moleculares, Dpto. de Bioquimica y Biolog\u00eda Molecular, Facultad de Ciencias Universidad Aut\u00f3noma  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Jimenez","sequence":"additional","affiliation":[{"name":"Centro de Diagn\u00f3stico de Enfermedades Moleculares, Dpto. de Bioquimica y Biolog\u00eda Molecular, Facultad de Ciencias Universidad Aut\u00f3noma  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Ugarte","sequence":"additional","affiliation":[{"name":"Centro de Diagn\u00f3stico de Enfermedades Moleculares, Dpto. de Bioquimica y Biolog\u00eda Molecular, Facultad de Ciencias Universidad Aut\u00f3noma  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Mart\u00ednez\u2010Pardo","sequence":"additional","affiliation":[{"name":"Departamento de Pediatria Centro Especial Ram\u00f3n y Cajal  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Lude\u00f1a","sequence":"additional","affiliation":[{"name":"Departamento de Pediatria Centro Especial Ram\u00f3n y Cajal  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Camarero","sequence":"additional","affiliation":[{"name":"Departamento de Pediatria Centro Especial Ram\u00f3n y Cajal  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"del Olmo","sequence":"additional","affiliation":[{"name":"Departamento de Pediatria Centro Especial Ram\u00f3n y Cajal  Madrid 34 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Duran","sequence":"additional","affiliation":[{"name":"University Children's Hospital, \u2018Het Whilhelmina Kinderziekenhuis\u2019  Nieuwe Gracht 137 Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S. K.","family":"Wadman","sequence":"additional","affiliation":[{"name":"University Children's Hospital, \u2018Het Whilhelmina Kinderziekenhuis\u2019  Nieuwe Gracht 137 Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1983,10,31]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198302000-00018"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(83)90186-9"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01810357"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(83)90246-2"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(76)90355-7"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(82)90361-8"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02312518"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.6857268"},{"key":"e_1_2_1_10_1","first-page":"263A","article-title":"Hypoketotic hypoglycemia \u2014 evidence for a new defect in fatty acid oxidation","volume":"16","author":"Stanley C. A.","year":"1982","journal-title":"Pediatr. Res."},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(79)90183-9"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF01805804","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T16:35:21Z","timestamp":1694882121000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/BF01805804"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1983,10,31]]},"references-count":10,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1984,6]]}},"alternative-id":["10.1007\/BF01805804"],"URL":"https:\/\/doi.org\/10.1007\/bf01805804","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1983,10,31]]}}}