{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T22:54:04Z","timestamp":1791327244543,"version":"4.3.1"},"reference-count":7,"publisher":"S. Karger AG","issue":"3","license":[{"start":{"date-parts":[[2009,11,4]],"date-time":"2009-11-04T00:00:00Z","timestamp":1257292800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2009,11,4]],"date-time":"2009-11-04T00:00:00Z","timestamp":1257292800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neonatology"],"published-print":{"date-parts":[[2010]]},"abstract":"<jats:p>In propionic aciduria and methylmalonic aciduria, hyperammonemia as a symptom of metabolic decompensation is one of the major clinical problems. Hyperammonemia is a true neonatal emergency with high mortality and neurological complications in most survivors. It requires a rapid and vigorous treatment in order to normalize the ammonia concentration as fast as possible. We report on two full-term neonates, one with propionic aciduria and the other with methylmalonic aciduria, whose plasma ammonia concentrations responded dramatically to oral &lt;i&gt;N&lt;\/i&gt;-carbamylglutamate. &lt;i&gt;N&lt;\/i&gt;-carbamylglutamate, added to the classic treatment, quickly normalized plasma ammonia levels in both patients and avoided the need of hemodialysis or peritoneal dialysis. A particularly sudden fall of ammonia was obtained in one patient through beginning &lt;i&gt;N&lt;\/i&gt;-carbamylglutamate treatment precociously.<\/jats:p>","DOI":"10.1159\/000255168","type":"journal-article","created":{"date-parts":[[2009,11,6]],"date-time":"2009-11-06T11:08:48Z","timestamp":1257505728000},"page":"286-290","source":"Crossref","is-referenced-by-count":52,"title":["&lt;i&gt;N&lt;\/i&gt;-Carbamylglutamate in Emergency Management of Hyperammonemia in Neonatal Acute Onset Propionic and Methylmalonic Aciduria"],"prefix":"10.1159","volume":"97","author":[{"given":"Luca","family":"Filippi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Gozzini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrizio","family":"Fiorini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Malvagia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giancarlo","family":"la Marca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Alice","family":"Donati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2009,11,4]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1172%2FJCI109614"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1023%2FA%3A1005393104494"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1016%2FS1096-7192%2803%2900095-7"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1007%2Fs10545-005-5260-7"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1186%2F1750-1172-3-2"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1007%2Fs00431-003-1347-z"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1203%2FPDR.0b013e318179454b"}],"container-title":["Neonatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/255168","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/255168","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T21:25:10Z","timestamp":1745443510000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000255168"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,11,4]]},"references-count":7,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2010,3,1]]}},"URL":"https:\/\/doi.org\/10.1159\/000255168","archive":["Portico"],"relation":{},"ISSN":["1661-7800","1661-7819"],"issn-type":[{"value":"1661-7800","type":"print"},{"value":"1661-7819","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,11,4]]}}}