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These compounds are found in tyrosinaemia and phenylketonuria but in our patients such disorders could not be established. By means of configuration analysis it was shown that these 2\u2010hydroxyacids consisted partly of the<jats:sc>D<\/jats:sc>\u2010enantiomers, pointing to a bacterial origin. Endogenously formed urinary 2\u2010hydroxyacids in tyrosinaemia or phenylketonuria consisted of only the<jats:sc>L<\/jats:sc>\u2010enantiomers. Furthermore, the urine of a patient with an established short bowel syndrome contained a wide variety of bacterial amino acid metabolites, including 2\u2010hydroxyisocaproic acid (2\u2010HICA). In this case 2\u2010HICA occurred predominantly in the<jats:sc>D<\/jats:sc>\u2010form whereas in the urine of a patient with maple syrup urine disease this compound appeared to have the<jats:sc>L<\/jats:sc>\u2010configuration.<\/jats:p>","DOI":"10.1007\/bf01799981","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T16:58:17Z","timestamp":1119373097000},"page":"383-390","source":"Crossref","is-referenced-by-count":29,"title":["Urinary D\u20104\u2010hydroxyphenyllactate, D\u2010phenyllactate and D\u20102\u2010hydroxyisocaproate, abnormalities of bacterial origin"],"prefix":"10.1002","volume":"10","author":[{"given":"L. J. M.","family":"Spaapen","sequence":"first","affiliation":[{"name":"Department of Genetics and Cell Biology University of Limburg  Beeldsnijdersdreef 101 Maastricht 6216 EA The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Ketting","sequence":"additional","affiliation":[{"name":"Het Wilhelmina Kinderziekenhuis University Children's Hospital  Nieuwe Gracht 137 Utrecht 3512 LK The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. 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