{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T12:58:09Z","timestamp":1762001889959,"version":"3.40.5"},"reference-count":30,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,6,11]],"date-time":"2020-06-11T00:00:00Z","timestamp":1591833600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JIMD Reports"],"published-print":{"date-parts":[[2020,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Hyperammonemic encephalopathy is a rare but potentially dangerous complication of the antiepileptic drug (AED) sodium valproate (VPA). We report a retrospective study of 25 pediatric patients, (15 females [60%]; age: 7.6 \u00b1\u20094.9\u2009years), with different underlying disorders, who suffered from hyperammonemia due to VPA and who were treated with carglumic acid (CA). The duration of treatment with VPA was 15\u2009\u00b1\u20091 month, with a dose of 40\u2009\u00b1\u200916.6 mg\/kg\/d. VPA blood levels were 75.5 \u00b1\u200960\u2009mg\/L with seven patients being overdosed (&gt;100\u2009mg\/L). Twenty\u2010three patients received concomitant treatment with other AEDs. The initial dose of CA was 100\u2009mg\/kg. Subsequently, CA doses of 25\u2009mg\/kg were given to 22 patients every 6 hours (average treatment length 2.17\u2009\u00b1\u20091.1\u2009days) until ammonemia was normalized. In nine patients, CA was used in combination with other drugs to treat hyperammonemia. In all cases, blood ammonia levels were brought under control and symptoms of hyperammonemia resolved. Two hours after CA administration, the average reduction in ammonium levels was 53\u2009\u00b1\u200929 and 88.6 \u00b1\u200947.5 \u03bcmol\/L at 24\u2009hours, resulting in a statistically significant decrease when compared to pretreatment levels. There were no statistically significant differences between sexes, in the presence or not of cognitive impairment or previous carnitine treatment. There were no statistically significant differences when comparing treatment with CA plus ammonia scavengers vs CA alone. In 17 patients (68%) VPA was discontinued and 62% of the patients who maintained treatment had recurrent episodes of hyperammonemia.<\/jats:p>","DOI":"10.1002\/jmd2.12131","type":"journal-article","created":{"date-parts":[[2020,6,12]],"date-time":"2020-06-12T06:36:36Z","timestamp":1591943796000},"page":"3-11","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Use of carglumic acid in valproate\u2010induced hyperammonemia: 25 pediatric cases"],"prefix":"10.1002","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2441-9311","authenticated-orcid":false,"given":"Laura Mar\u00eda","family":"Palomino P\u00e9rez","sequence":"first","affiliation":[{"name":"Section of Gastroenterology and Nutrition Hospital Infantil Universitario Ni\u00f1o Jes\u00fas  Madrid 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