{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T15:32:52Z","timestamp":1786980772362,"version":"build-2736575974"},"reference-count":7,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2012,12,19]],"date-time":"2012-12-19T00:00:00Z","timestamp":1355875200000},"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":[[2013,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Acute decompensation of maple syrup urine disease (MSUD) is usually treated by enteral feeding with an amino\u2010acid mixture without leucine (Leu), valine or isoleucine. However, its administration is ineffective in cases of gastric intolerance and some adult patients refuse enteral feeding via a nasogastric tube. We developed a new parenteral amino\u2010acid mixture for patients with MSUD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Seventeen decompensation episodes in four adult patients with MSUD treated with a parenteral amino\u2010acid mixture (group P) were compared to 18 previous episodes in the same patients treated by enteral feeding (group E).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The mean Leu concentration at presentation was similar in the groups P and E (1196.9 \u03bcmol\/L and 1212.2 \u03bcmol\/L, respectively). The mean decrease in the Leu concentration during the first 3 days of hospitalisation was significantly higher in group P than group E (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0026); there were no side effects. The mean duration of hospitalisation was similar (4 vs. 4.5 days,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009NS). No patient in group P deteriorated whereas one patient in group E required dialysis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This new parenteral amino\u2010acid mixture is safe and allows efficient Leu concentration decrease during acute MSUD decompensation episodes in adults. Its use avoids the need for nasogastric tube insertion.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s10545-012-9570-2","type":"journal-article","created":{"date-parts":[[2012,12,18]],"date-time":"2012-12-18T05:20:52Z","timestamp":1355808052000},"page":"939-944","source":"Crossref","is-referenced-by-count":17,"title":["Treatment of acute decompensation of maple syrup urine disease in adult patients with a new parenteral amino\u2010acid mixture"],"prefix":"10.1002","volume":"36","author":[{"given":"A.","family":"Servais","sequence":"first","affiliation":[{"name":"Department of Nephrology H\u00f4pital Necker\u2010Enfants Malades AP\u2010HP Paris France"},{"name":"Universit\u00e9 Paris Descartes Paris France"},{"name":"Reference Center for Inherited Metabolic Disorders H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"},{"name":"Service de N\u00e9phrologie adultes H\u00f4pital Necker 149 rue de S\u00e8vres Paris 75015 France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. B.","family":"Arnoux","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris Descartes Paris France"},{"name":"Reference Center for Inherited Metabolic Disorders H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Lamy","sequence":"additional","affiliation":[{"name":"Department of Pharmacy H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Hummel","sequence":"additional","affiliation":[{"name":"Department of Nephrology H\u00f4pital Necker\u2010Enfants Malades AP\u2010HP Paris France"},{"name":"Universit\u00e9 Paris Descartes Paris France"},{"name":"Reference Center for Inherited Metabolic Disorders H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Vittoz","sequence":"additional","affiliation":[{"name":"Department of Nephrology H\u00f4pital Necker\u2010Enfants Malades AP\u2010HP Paris France"},{"name":"Universit\u00e9 Paris Descartes Paris France"},{"name":"Reference Center for Inherited Metabolic Disorders H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"Katerinis","sequence":"additional","affiliation":[{"name":"Department of Nephrology H\u00f4pital Necker\u2010Enfants Malades AP\u2010HP Paris France"},{"name":"Universit\u00e9 Paris Descartes Paris France"},{"name":"Reference Center for Inherited Metabolic Disorders H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.","family":"Bazzaoui","sequence":"additional","affiliation":[{"name":"Reference Center for Inherited Metabolic Disorders H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"},{"name":"Department of Dietetic H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Dubois","sequence":"additional","affiliation":[{"name":"Reference Center for Inherited Metabolic Disorders H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"},{"name":"Department of Dietetic H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Broissand","sequence":"additional","affiliation":[{"name":"Department of Pharmacy H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. C.","family":"Husson","sequence":"additional","affiliation":[{"name":"AGEPS, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. P.","family":"Berleur","sequence":"additional","affiliation":[{"name":"AGEPS, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Rabier","sequence":"additional","affiliation":[{"name":"Department of Biochemistry H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Ottolenghi","sequence":"additional","affiliation":[{"name":"Department of Biochemistry H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.","family":"Valayannopoulos","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris Descartes Paris France"},{"name":"Reference Center for Inherited Metabolic Disorders H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"de Lonlay","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris Descartes Paris France"},{"name":"Reference Center for Inherited Metabolic Disorders H\u00f4pital Necker\u2010Enfants Malades, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,12,19]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199101173240307"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1203\/01.PDR.0000169982.06717.67"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.152.6.593"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-642-15720-2_19"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1987.tb01039.x"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2009.12.007"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/36.2.359"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-012-9570-2","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T03:36:23Z","timestamp":1694748983000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-012-9570-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,12,19]]},"references-count":7,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2013,11]]}},"alternative-id":["10.1007\/s10545-012-9570-2"],"URL":"https:\/\/doi.org\/10.1007\/s10545-012-9570-2","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,12,19]]}}}