{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T10:08:16Z","timestamp":1790676496269,"version":"4.1.0"},"reference-count":25,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2022,7,2]],"date-time":"2022-07-02T00:00:00Z","timestamp":1656720000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Recordati Rare Diseases","award":["APC"],"award-info":[{"award-number":["APC"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Hyperammonaemia is a metabolic derangement that may cause severe neurological damage and even death due to cerebral oedema, further complicating the prognosis of its triggering disease. In small children it is a rare condition usually associated to inborn errors of the metabolism. As age rises, and especially in adults, it may be precipitated by heterogeneous causes such as liver disease, drugs, urinary infections, shock, or dehydration. In older patients, it is often overlooked, or its danger minimized. This protocol was drafted to provide an outline of the clinical measures required to normalise ammonia levels in patients of all ages, aiming to assist clinicians with no previous experience in its treatment. It is an updated protocol developed by a panel of experts after a review of recent publications. We point out the importance of frequent monitoring to assess the response to treatment, the nutritional measures that ensure not only protein restriction but adequate caloric intake and the need to avoid delays in the use of specific pharmacological therapies and, especially, extrarenal clearance measures. In this regard, we propose initiating haemodialysis when ammonia levels are &gt;200\u2013350 \u00b5mol\/L in children up to 18 months of age and &gt;150\u2013200 \u00b5mol\/L after that age.<\/jats:p>","DOI":"10.3390\/nu14132755","type":"journal-article","created":{"date-parts":[[2022,7,4]],"date-time":"2022-07-04T20:59:18Z","timestamp":1656968358000},"page":"2755","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["Recommendations for the Diagnosis and Therapeutic Management of Hyperammonaemia in Paediatric and Adult Patients"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0102-8956","authenticated-orcid":false,"given":"Amaya","family":"B\u00e9langer-Quintana","sequence":"first","affiliation":[{"name":"Department of Paediatric Inborn Errors of Metabolism, Hospital Universitario Ram\u00f3n y Cajal, Carretera de Colmenar Viejo, Km. 9, 100, 28034 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisco","family":"Arrieta Blanco","sequence":"additional","affiliation":[{"name":"Department of Adult Inborn Errors of Metabolism, Hospital Universitario Ram\u00f3n y Cajal, Carretera de Colmenar Viejo, Km. 9, 100, 28034 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delia","family":"Barrio-Carreras","sequence":"additional","affiliation":[{"name":"Department of Inborn Errors of Metabolism, Hospital Universitario 12 de Octubre, Avenida de C\u00f3rdoba, s\/n, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Bergua Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Department of Paediatric Nutrition and Metabolic Diseases, Hospital Universitario La Paz, Paseo de la Castellana, 261, 28046 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elvira","family":"Ca\u00f1edo Villarroya","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Nutrition, Hospital Universitario Ni\u00f1o Jes\u00fas, Avenida de Men\u00e9ndez Pelayo, 65, 28009 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda Teresa","family":"Garc\u00eda-Silva","sequence":"additional","affiliation":[{"name":"Department of Inborn Errors of Metabolism, Hospital Universitario 12 de Octubre, Avenida de C\u00f3rdoba, s\/n, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosa","family":"Lama More","sequence":"additional","affiliation":[{"name":"Department of Paediatric Nutrition, Centro M\u00e9dico D-Medical, Calle del Pr\u00edncipe de Vergara, 44, 28001 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Mart\u00edn-Hern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Department of Inborn Errors of Metabolism, Hospital Universitario 12 de Octubre, Avenida de C\u00f3rdoba, s\/n, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Mor\u00e1is","family":"L\u00f3pez","sequence":"additional","affiliation":[{"name":"Department of Paediatric Nutrition and Metabolic Diseases, Hospital Universitario La Paz, Paseo de la Castellana, 261, 28046 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Montserrat","family":"Morales-Conejo","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Hospital Universitario 12 de Octubre, Avenida de C\u00f3rdoba, s\/n, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Consuelo","family":"Pedr\u00f3n-Giner","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Nutrition, Hospital Universitario Ni\u00f1o Jes\u00fas, Avenida de Men\u00e9ndez Pelayo, 65, 28009 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1026-4854","authenticated-orcid":false,"given":"Pilar","family":"Quijada-Fraile","sequence":"additional","affiliation":[{"name":"Department of Inborn Errors of Metabolism, Hospital Universitario 12 de Octubre, Avenida de C\u00f3rdoba, s\/n, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0340-4580","authenticated-orcid":false,"given":"Sinziana","family":"Stanescu","sequence":"additional","affiliation":[{"name":"Department of Paediatric Inborn Errors of Metabolism, Hospital Universitario Ram\u00f3n y Cajal, Carretera de Colmenar Viejo, Km. 9, 100, 28034 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mercedes Mart\u00ednez-Pardo","family":"Casanova","sequence":"additional","affiliation":[{"name":"Department of Paediatric Inborn Errors of Metabolism, Hospital Universitario Ram\u00f3n y Cajal, Carretera de Colmenar Viejo, Km. 9, 100, 28034 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,2]]},"reference":[{"key":"ref_1","unstructured":"Adam, M.P., Ardinger, H.H., Pagon, R.A., Wallace, S.E., Bean, L.J.H., Stephens, K., and Amemiya, A. 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