{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T01:47:10Z","timestamp":1773280030679,"version":"3.50.1"},"reference-count":25,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2020,5,28]],"date-time":"2020-05-28T00:00:00Z","timestamp":1590624000000},"content-version":"vor","delay-in-days":27,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatrics International"],"published-print":{"date-parts":[[2020,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Acute liver failure (ALF) is a rare multisystemic disease occurring in individuals with no history of liver disease, characterized by coagulopathy and \/ or hepatic encephalopathy secondary to acute liver injury. It is mostly caused by viral infections, drug intoxication, and metabolic diseases (MD), and can also have an indeterminate etiology. In this study, we aimed to evaluate the demographic and clinical characteristics and clinical outcomes of the patients that presented to our clinic with MD\u2010associated ALF.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective study reviewed age, gender, parental consanguinity, family history, presence of encephalopathy, laboratory parameters, and clinical outcomes of the patients that presented to our clinic between January 2009 and January 2019. Patients with MD\u2010associated ALF were compared with patients in whom ALF was associated with other etiologies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The study included 39 patients (53.8% boys; mean age\u00a0+\u00a0SD 6.13\u00a0\u00b1\u00a01.43\u00a0years). The total and direct bilirubin, international normalized ratio, and ammoniac levels were significantly higher in patients with MD than in the others (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.05). Moreover, the incidences of hypoglycemia, death of a sibling and \/ or a family history of liver disease were also higher in patients with MD than in the others (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.05). On the other hand, alanine aminotransferase (ALT) levels were significantly higher in patients with other etiologies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Metabolic diseases should be kept in mind in patients with a history of parental consanguinity and a positive family history of liver disease\u00a0along with less increased alanine aminotransferase\u00a0than expected, and increased bilirubin, international normalized ratio, and ammoniac levels and hypoglycemia. As the number of these parameters increases, the chance of diagnosis increases.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ped.14230","type":"journal-article","created":{"date-parts":[[2020,3,14]],"date-time":"2020-03-14T13:59:28Z","timestamp":1584194368000},"page":"609-614","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Acute liver failure associated with metabolic diseases: A 10\u2010year single\u2010center experience"],"prefix":"10.1111","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5142-8168","authenticated-orcid":false,"given":"Burcu","family":"G\u00fcven","sequence":"first","affiliation":[{"name":"Department of Pediatric Gastroenterology Karadeniz Technical University Faculty of Medicine Trabzon Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elif","family":"Sa\u011f","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology Karadeniz Technical University Faculty of Medicine Trabzon Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00fclay","family":"Karag\u00fczel","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology Karadeniz Technical University Faculty of Medicine Trabzon Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Murat","family":"\u00c7ak\u0131r","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology Karadeniz Technical University Faculty of Medicine Trabzon Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,5,28]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.13464"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13312-016-0913-1"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2001.119989"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2011.04.016"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.12.051"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1665-2681(19)31321-3"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinre.2012.03.022"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2015.03.057"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000001178"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/NMC.0000000000000388"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.21037\/atm.2018.12.53"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2017.101"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1159\/000448321"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/s10038-019-0614-4"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01831.x"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jceh.2013.10.004"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2008-1073115"},{"key":"e_1_2_8_19_1","first-page":"1","article-title":"Clinical and biochemical footprints of inherited metabolic diseases. 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