{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T19:37:55Z","timestamp":1783971475452,"version":"3.55.0"},"reference-count":0,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2016,1,1]],"date-time":"2016-01-01T00:00:00Z","timestamp":1451606400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2016,1,1]],"date-time":"2016-01-01T00:00:00Z","timestamp":1451606400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Blood Purif"],"published-print":{"date-parts":[[2016]]},"abstract":"<jats:p>&lt;b&gt;&lt;i&gt;Background:&lt;\/i&gt;&lt;\/b&gt; The study aims to define the efficacy of continuous renal replacement therapy in acute metabolic decompensation treatment of maple syrup urine disease (MSUD). &lt;b&gt;&lt;i&gt;Methods:&lt;\/i&gt;&lt;\/b&gt; All the neonates, infants and children who have had life threatening conditions due to MSUD and were treated with continuous venovenous hemodiafiltration (CVVHDF) were analyzed retrospectively. &lt;b&gt;&lt;i&gt;Results:&lt;\/i&gt;&lt;\/b&gt; Fourteen patients underwent 15 sessions of CVVHDF (age range 15 days to 87 months, mean 40.8 \u00b1 31.4 months). One patient required additional CVVHDF 1 week after cessation of CVVHDF. Twenty seven percent (n = 4) of the patients were intubated and mechanically ventilated. Twelve patients responded to treatment and dramatic neurological improvement was observed within 24 h. Two of the 14 patients required 36 h of CVVHDF for neurological improvement. The mean duration of CVVHDF was 20.2 \u00b1 8.6 (9-36) h. The mean leucine level was 1,648 \u00b1 623.8 (714-2,768) \u03bcmol\/l before and was 256.5 \u00b1 150.6 (117-646) \u03bcmol\/l at the end of treatment. No mortality was observed. &lt;b&gt;&lt;i&gt;Conclusion:&lt;\/i&gt;&lt;\/b&gt; Continuous hemodiafiltration is an effective and safe method in correcting metabolic disturbances in MSUD.<\/jats:p>","DOI":"10.1159\/000443783","type":"journal-article","created":{"date-parts":[[2016,3,22]],"date-time":"2016-03-22T13:29:42Z","timestamp":1458653382000},"page":"27-32","source":"Crossref","is-referenced-by-count":11,"title":["Continuous Venovenous Hemodiafiltration in the Treatment of Maple Syrup Urine Disease"],"prefix":"10.1159","volume":"42","author":[{"given":"Demet","family":"Demirkol","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00fcnt\u00fcl\u00fc","family":"\u015e\u0131k","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nil\u00fcfer","family":"Topal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agop","family":"\u00c7\u0131tak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c7igdem","family":"Zeybek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abd\u00fclhamit","family":"T\u00fcten","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilmay","family":"Bilge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2016,3,22]]},"container-title":["Blood Purification"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/443783","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/443783","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T17:41:08Z","timestamp":1745430068000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000443783"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016]]},"references-count":0,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2016,6,6]]}},"URL":"https:\/\/doi.org\/10.1159\/000443783","archive":["Portico"],"relation":{},"ISSN":["0253-5068","1421-9735"],"issn-type":[{"value":"0253-5068","type":"print"},{"value":"1421-9735","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016]]}}}