{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T09:45:34Z","timestamp":1767174334189,"version":"build-2238731810"},"reference-count":10,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,6,21]],"date-time":"2024-06-21T00:00:00Z","timestamp":1718928000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,6,21]],"date-time":"2024-06-21T00:00:00Z","timestamp":1718928000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["JA Clin Rep"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare autosomal recessive urea cycle disorder associated with a high risk of exacerbation of hyperammonemia during the perioperative period. Here, we describe an adult patient with HHH syndrome who developed hyperammonemic encephalopathy secondary to postoperative constipation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>A 52-year-old patient with HHH syndrome underwent intrathecal baclofen pump insertion for lower limb spasticity under general anesthesia. The surgery was uneventful, without any increase in serum ammonia levels. However, after surgery, he was constipated, and on postoperative day (POD) 3, he fell into a coma with an exacerbation of hyperammonemia (894\u00a0\u00b5g\/dL). After administering a glycerin enema, he defecated, leading to a rapid decrease in serum ammonia levels to 165\u00a0\u00b5g\/dL. He regained consciousness, and serum ammonia levels remained stable as long as he defecated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>We suggest strict management of defecation during the perioperative period to prevent hyperammonemia in patients with HHH syndrome.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s40981-024-00726-z","type":"journal-article","created":{"date-parts":[[2024,6,21]],"date-time":"2024-06-21T01:01:39Z","timestamp":1718931699000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Postoperative hyperammonemic encephalopathy due to unexpected constipation in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: a case report"],"prefix":"10.1186","volume":"10","author":[{"given":"Haruka","family":"Tachibana","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobuhiko","family":"Ohashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gaku","family":"Okumura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9221-2486","authenticated-orcid":false,"given":"Ryusuke","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Fuseya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sayako","family":"Gotoh","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takashi","family":"Ishida","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sari","family":"Shimizu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mikito","family":"Kawamata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,6,21]]},"reference":[{"key":"726_CR1","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1186\/s13023-015-0242-9","volume":"10","author":"D Martinelli","year":"2015","unstructured":"Martinelli D, Diodato D, Ponzi E, Monn\u00e9 M, Boenzi S, Bertini E, et al. The hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. Orphanet J Rare Dis. 2015;10:29.","journal-title":"Orphanet J Rare Dis"},{"key":"726_CR2","doi-asserted-by":"publisher","first-page":"1192","DOI":"10.1002\/jimd.12100","volume":"42","author":"J H\u00e4berle","year":"2019","unstructured":"H\u00e4berle J, Burlina A, Chakrapani A, Dixon M, Karall D, Lindner M, et al. Suggested guidelines for the diagnosis and management of urea cycle disorders: first revision. J Inherit Metab Dis. 2019;42:1192\u2013230.","journal-title":"J Inherit Metab Dis"},{"key":"726_CR3","doi-asserted-by":"publisher","first-page":"303","DOI":"10.1007\/s007260170016","volume":"21","author":"J M\u00fchling","year":"2001","unstructured":"M\u00fchling J, Dehne MG, Fuchs M, Sablotzki A, Weiss S, Spatz J, et al. Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesia. Amino Acids. 2001;21:303\u201318.","journal-title":"Amino Acids"},{"key":"726_CR4","first-page":"86","volume":"37","author":"R Noguchi","year":"1988","unstructured":"Noguchi R, Tomita Y, Hashiba M, Shimoji K. Anesthesia for a patient with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. Masui. 1988;37:86\u201392.","journal-title":"Masui"},{"key":"726_CR5","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1007\/s11940-014-0297-2","volume":"16","author":"JA Frontera","year":"2014","unstructured":"Frontera JA. Management of hepatic encephalopathy. Curr Treat Options Neurol. 2014;16:297.","journal-title":"Curr Treat Options Neurol"},{"key":"726_CR6","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1001\/archpedi.1969.02100030085009","volume":"117","author":"VE Shir","year":"1969","unstructured":"Shir VE, Efron ML, Moser HW. Hyperornithinemia, hyperammonemia, and homocitrullinuria. A new disorder of amino acid metabolism associated with myoclonic seizures and mental retardation. Am J Dis Child. 1969;117:83\u201392.","journal-title":"Am J Dis Child."},{"key":"726_CR7","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1093\/bja\/85.1.109","volume":"85","author":"JP Desborough","year":"2000","unstructured":"Desborough JP. The stress response to trauma and surgery. Br J Anaesth. 2000;85:109\u201317.","journal-title":"Br J Anaesth"},{"key":"726_CR8","doi-asserted-by":"publisher","first-page":"780","DOI":"10.1111\/pan.13905","volume":"30","author":"C Del R\u00edo","year":"2020","unstructured":"Del R\u00edo C, Mart\u00edn-Hern\u00e1ndez E, Ruiz A, Quijada-Fraile P, Rubio P. Perioperative management of children with urea cycle disorders. Paediatr Anaesth. 2020;30:780\u201391.","journal-title":"Paediatr Anaesth"},{"key":"726_CR9","doi-asserted-by":"publisher","first-page":"193","DOI":"10.2147\/CEG.S160921","volume":"11","author":"DG Levitt","year":"2018","unstructured":"Levitt DG, Levitt MD. A model of blood-ammonia homeostasis based on a quantitative analysis of nitrogen metabolism in the multiple organs involved in the production, catabolism, and excretion of ammonia in humans. Clin Exp Gastroenterol. 2018;11:193\u2013215.","journal-title":"Clin Exp Gastroenterol"},{"key":"726_CR10","first-page":"e6678","volume":"12","author":"M Sharma","year":"2020","unstructured":"Sharma M, Anjum H, Bulathsinghala CP, De Silva PR, Surani S. An intriguing case of acute encephalopathy: lesson learned from a constipated man. Cureus. 2020;12:e6678.","journal-title":"Cureus"}],"updated-by":[{"DOI":"10.1186\/s40981-024-00753-w","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2024,11,14]],"date-time":"2024-11-14T00:00:00Z","timestamp":1731542400000}}],"container-title":["JA Clinical Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40981-024-00726-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s40981-024-00726-z\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40981-024-00726-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,14]],"date-time":"2024-11-14T03:03:00Z","timestamp":1731553380000},"score":1,"resource":{"primary":{"URL":"https:\/\/jaclinicalreports.springeropen.com\/articles\/10.1186\/s40981-024-00726-z"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,21]]},"references-count":10,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["726"],"URL":"https:\/\/doi.org\/10.1186\/s40981-024-00726-z","relation":{},"ISSN":["2363-9024"],"issn-type":[{"value":"2363-9024","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,21]]},"assertion":[{"value":"1 May 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 June 2024","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 June 2024","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 June 2024","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 November 2024","order":5,"name":"change_date","label":"Change Date","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Update","order":6,"name":"change_type","label":"Change Type","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The original online version of this article was revised: Two author were added.","order":7,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 November 2024","order":8,"name":"change_date","label":"Change Date","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Correction","order":9,"name":"change_type","label":"Change Type","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"A Correction to this paper has been published:","order":10,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"https:\/\/doi.org\/10.1186\/s40981-024-00753-w","URL":"https:\/\/doi.org\/10.1186\/s40981-024-00753-w","order":11,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent form is available for review by the editor-in-chief of the journal.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare they have no conflicts of interest.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"42"}}