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With 1:35\u2009000 estimated incidence, UCDs cause hyperammonemia of neonatal (~50%) or late onset that can lead to intellectual disability or death, even while effective therapies do exist. In the 7 years that have elapsed since the first guideline was published, abundant novel information has accumulated, experience on newborn screening for some UCDs has widened, a novel hyperammonemia\u2010causing genetic disorder has been reported, glycerol phenylbutyrate has been introduced as a treatment, and novel promising therapeutic avenues (including gene therapy) have been opened. Several factors including the impact of the first edition of these guidelines (frequently read and quoted) may have increased awareness among health professionals and patient families. However, under\u2010recognition and delayed diagnosis of UCDs still appear widespread. It was therefore necessary to revise the original guidelines to ensure an up\u2010to\u2010date frame of reference for professionals and patients as well as for awareness campaigns. This was accomplished by keeping the original spirit of providing a trans\u2010European consensus based on robust evidence (scored with GRADE methodology), involving professionals on UCDs from nine countries in preparing this consensus. We believe this revised guideline, which has been reviewed by several societies that are involved in the management of UCDs, will have a positive impact on the outcomes of patients by establishing common standards, and spreading and harmonizing good practices. It may also promote the identification of knowledge voids to be filled by future research.<\/jats:p>","DOI":"10.1002\/jimd.12100","type":"journal-article","created":{"date-parts":[[2019,4,15]],"date-time":"2019-04-15T02:41:16Z","timestamp":1555296076000},"page":"1192-1230","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":480,"title":["Suggested guidelines for the diagnosis and management of urea cycle disorders: First revision"],"prefix":"10.1002","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0635-091X","authenticated-orcid":false,"given":"Johannes","family":"H\u00e4berle","sequence":"first","affiliation":[{"name":"University Children's Hospital Zurich and Children's Research Centre  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Burlina","sequence":"additional","affiliation":[{"name":"Division of Inborn Metabolic Disease, Department of Pediatrics 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