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The aging of UCD patients leads to a better knowledge of the long\u2010term natural history of the condition and to the reporting of previously unnoticed manifestations. Despite historical evidence of liver involvement in UCDs, little attention has been paid to this organ until recently. Hence, we reviewed the available scientific evidence on acute and chronic liver dysfunction and liver carcinogenesis in UCDs and discuss their pathophysiology. Overall, liver involvement, such as acute liver failure or steatotic\u2010like disease, which may evolve toward cirrhosis, has been reported in all six main UCDs. Excessive glycogen storage is also a prominent histologic feature, and hypoglycemia has been reported in citrin deficiency. Hepatocarcinomas seem frequent in some UCDs, such as in citrin deficiency, and can sometimes occur in non\u2010cirrhotic patients. UCDs may differ in liver involvement according to the enzymatic deficiency. Ornithine transcarbamylase deficiency may be associated more with acute liver failure and argininosuccinic aciduria with chronic liver failure and cirrhosis. Direct toxicity of metabolites, downstream metabolic deficiencies, impaired tricarboxylic acid cycle, oxidative stress, mitochondrial dysfunction, energy deficit, and putative toxicity of therapies combine in various ways to cause the different liver diseases reported.<\/jats:p>","DOI":"10.1007\/s10545-017-0088-5","type":"journal-article","created":{"date-parts":[[2017,9,12]],"date-time":"2017-09-12T11:17:39Z","timestamp":1505215059000},"page":"757-769","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Liver involvement in urea cycle disorders: a review of the literature"],"prefix":"10.1002","volume":"40","author":[{"given":"Adrien","family":"Bigot","sequence":"first","affiliation":[{"name":"CHRU de Tours, service m\u00e9decine interne Tours France"},{"name":"CHRU de Tours, centre de r\u00e9f\u00e9rence des maladies h\u00e9r\u00e9ditaires du m\u00e9tabolisme Tours France"},{"name":"Department of Genetic 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