{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T10:07:35Z","timestamp":1761127655615},"reference-count":19,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[1997,8,1]],"date-time":"1997-08-01T00:00:00Z","timestamp":870393600000},"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":[[1997,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Maple syrup urine disease results in accumulation of leucine and its metabolites, which may lead in the long term to neurological dysfunction. In acute neonatal crises, large amounts of leucine may be removed by continuous venovenous haemofiltration. This extracorporeal technique has its risks and hazards, which increase with duration of treatment. We report three neonates in life\u2010threatening conditions due to maple syrup urine disease, treated for not more than 12h with various continuous venovenous techniques: continuous haemofiltration, haemodiafiltration and haemodialysis. The efficiency of and tolerance to these techniques was evaluated. For all three patients, plasma leucine levels decreased dramatically from 2186, 3818 and 2536 \u00b5mol\/L to 1131, 1275 and 488 \u00b5mol\/L, respectively. Leucine clearance obtained was 4.28 ml\/min in haemodiafiltration. Their patients' neurological status improved rapidly and they have a normal developmental quotient at 22 months, 13 months, and 11 months of age, respectively. Tolerance was good except for hypothermia and drop in haematocrit in all cases. Haemodiafiltration management was more cumbersome and time consuming because it required continual adjustment of the substitution fluid flow rate to precisely balance inflow and outflow rates. We recommend continuous venovenous haemodialysis as the therapy of choice. It might be anticipated that improvement of this technique, by increasing dialysate flow rate and blood flow rate, will allow leucine concentration to be decreased below 1000 \u00b5mol\/L within 6\u20108 h, whatever the initial level.<\/jats:p>","DOI":"10.1023\/a:1005314025760","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T21:48:34Z","timestamp":1040507314000},"page":"463-472","source":"Crossref","is-referenced-by-count":42,"title":["Continuous venovenous haemodiafiltration in the acute phase of neonatal maple syrup urine disease"],"prefix":"10.1002","volume":"20","author":[{"given":"P.","family":"Jouvet","sequence":"first","affiliation":[{"name":"Pediatric Medical and Surgical Department, INSERM U90 H\u00f4pital Necker Enfants Malades  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F.","family":"Poggi","sequence":"additional","affiliation":[{"name":"Pediatric Medical and Surgical Department, INSERM U90 H\u00f4pital Necker Enfants Malades  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Rabier","sequence":"additional","affiliation":[{"name":"Biochemistry Department, INSERM U90 H\u00f4pital Necker Enfants Malades  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. L.","family":"Michel","sequence":"additional","affiliation":[{"name":"Biochemistry Department, INSERM U90 H\u00f4pital Necker Enfants Malades  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Hubert","sequence":"additional","affiliation":[{"name":"Pediatric Medical and Surgical Department, INSERM U90 H\u00f4pital Necker Enfants Malades  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Sposito","sequence":"additional","affiliation":[{"name":"Nephrology Department, INSERM U90 H\u00f4pital Necker Enfants Malades  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. M.","family":"Saudubray","sequence":"additional","affiliation":[{"name":"Pediatric Medical and Surgical Department, INSERM U90 H\u00f4pital Necker Enfants Malades  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N. K.","family":"Man","sequence":"additional","affiliation":[{"name":"Nephrology Department, INSERM U90 H\u00f4pital Necker Enfants Malades  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1997,8]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00858687"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0272-6386(95)90618-5"},{"key":"e_1_2_1_4_1","first-page":"803","article-title":"Hemofiltration art\u00e9rioveineuse continue: prise en charge en cas d'une leucinose n\u00e9onatale","volume":"49","author":"Casadevall I","year":"1992","journal-title":"Arch Fr Pediatr"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00866350"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4684-2673-1_2"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199403000-00017"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00858994"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)81037-6"},{"key":"e_1_2_1_10_1","first-page":"176","article-title":"Renal clearance of branched chain 2\u2010oxo\u2010acids in maple syrup urine disease","volume":"17","author":"Langenbeck U","year":"1979","journal-title":"J Clin Chem Clin Biochem"},{"key":"e_1_2_1_11_1","first-page":"192","article-title":"Effects of alterations of oxygen transport on the neonate","volume":"8","author":"Lister G","year":"1984","journal-title":"Semin Perinatol"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-662-03147-6_3"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(19)34609-4"},{"key":"e_1_2_1_14_1","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1542\/peds.92.2.280","article-title":"Nasogastric drip feeding as the only treatment of neonatal maple syrup urine disease","volume":"92","author":"Parini R","year":"1993","journal-title":"Pediatrics"},{"key":"e_1_2_1_15_1","first-page":"187","article-title":"Continuous renal replacement therapies for the treatment of acute renal failure in intensive care patients","volume":"40","author":"Ronco C","year":"1993","journal-title":"Clin Nephrol"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF03047365"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1442-200X.1988.tb02531.x"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)82198-5"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198112000-00009"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/00003246-198707000-00014"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1023%2FA%3A1005314025760","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1023\/A%3A1005314025760","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T04:31:26Z","timestamp":1694752286000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1023\/A%3A1005314025760"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1997,8]]},"references-count":19,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1997,8]]}},"alternative-id":["10.1023\/A:1005314025760"],"URL":"https:\/\/doi.org\/10.1023\/a:1005314025760","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1997,8]]}}}