{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,30]],"date-time":"2026-05-30T00:18:07Z","timestamp":1780100287561,"version":"3.54.0"},"reference-count":12,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[1992,10,8]],"date-time":"1992-10-08T00:00:00Z","timestamp":718502400000},"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":[[1993,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>An infant with glycogen storage disease and prolonged malnourishment showed a urinary organic acid profile during an episode of fasting hypoglycaemia with inappropriate hypoketotic dicarboxylic aciduria that was indistinguishable from that reported in long\u2010chain<jats:sc>l<\/jats:sc>\u20103\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency. Although there was a striking elevation of urinary 3\u2010hydroxydecanedioic acid, the ratios between hydroxydicarboxylic acids were consistent with values reported to be indicative of medium\u2010chain acyl\u2010CoA dehydrogenase deficiency. We suspect that the fasting 3\u2010hydroxydicarboxylic aciduria was attributable to secondarily impaired enzyme activities, the consequence of malnutrition, early infancy, and\/or glycogen storage disease. Caution is advised in the interpretation of urinary organic acid patterns that indicate a 3\u2010hydroxydicarboxylic aciduria, as well as an inapprorpiate hypoketotic dicarboxylic aciduria, as they may represent non\u2010specific findings.<\/jats:p>","DOI":"10.1007\/bf00714277","type":"journal-article","created":{"date-parts":[[2004,12,2]],"date-time":"2004-12-02T03:42:42Z","timestamp":1101958962000},"page":"851-856","source":"Crossref","is-referenced-by-count":16,"title":["Marked elevation of urinary 3\u2010hydroxydecanedioic acid in a malnourished infant with glycogen storage disease, mimicking long\u2010chain<scp>l<\/scp>\u20103\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency"],"prefix":"10.1002","volume":"16","author":[{"given":"J.","family":"Bergoffen","sequence":"first","affiliation":[{"name":"Division of Human Genetics and Molecular Biology The Children's Hospital of Philadelphia  34th Street and Civic Center Boulevard Philadelphia PA 19104 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Kaplan","sequence":"additional","affiliation":[{"name":"Division of Human Genetics and Molecular Biology The Children's Hospital of Philadelphia  34th Street and Civic Center Boulevard Philadelphia PA 19104 USA"},{"name":"Division of Biochemical Development and Molecular Diseases The Children's Hospital of Philadelphia  34th Street and Civic Center Boulevard Philadelphia PA 19104 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. E.","family":"Hale","sequence":"additional","affiliation":[{"name":"Division of Endocrinology and Diabetes The Children's Hospital of Philadelphia  34th Street and Civic Center Boulevard Philadelphia PA 19104 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. J.","family":"Bennett","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Nutrition The Children's Hospital of Philadelphia  34th Street and Civic Center Boulevard Philadelphia PA 19104 USA"},{"name":"Metabolic Disease Center Baylor University Medical Center  3500 Gaston Avenue Dallas TX 75246 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. T.","family":"Berry","sequence":"additional","affiliation":[{"name":"Division of Biochemical Development and Molecular Diseases The Children's Hospital of Philadelphia  34th Street and Civic Center Boulevard Philadelphia PA 19104 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1992,10,8]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1177\/000456329202900509"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(74)90065-5"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/26.2.261"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)82826-4"},{"key":"e_1_2_1_6_1","first-page":"503","article-title":"Thel\u20103\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency","volume":"321","author":"Hale DE","year":"1990","journal-title":"Prog Clin Biol Res"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(88)90031-9"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01800227"},{"key":"e_1_2_1_9_1","first-page":"495","volume-title":"Fatty Acid Oxidation Clinical, Biochemical, and Molecular Aspects","author":"Pollitt PJ","year":"1990"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799932"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799734"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199012000-00023"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0026-0495(91)90083-9"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF00714277","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF00714277","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T12:50:42Z","timestamp":1694868642000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/BF00714277"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1992,10,8]]},"references-count":12,"journal-issue":{"issue":"5","published-print":{"date-parts":[[1993,9]]}},"alternative-id":["10.1007\/BF00714277"],"URL":"https:\/\/doi.org\/10.1007\/bf00714277","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1992,10,8]]}}}