{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,1,19]],"date-time":"2024-01-19T00:10:10Z","timestamp":1705623010630},"reference-count":10,"publisher":"American Dental Society of Anesthesiology (ADSA)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,10,1]]},"abstract":"<jats:p>We report a case involving intravenous sedation for third molar extractions in a 32-year-old man with citrullinemia type I (CTLN1), a genetic disorder that affects the urea cycle. The patient was diagnosed with CTLN1 after he exhibited seizures soon after birth and was intellectually disabled because of persistent hyperammonemia, although his recent serum ammonia levels were fairly well controlled. We planned to minimize his preoperative fasting, continue his routine oral medications, and monitor his serum ammonia levels at least twice. Sedation with midazolam and a propofol infusion was planned to suppress his gag reflex and reduce protein hypercatabolism due to stress. Epinephrine-containing local anesthetics, which enhance protein catabolism, were avoided, replaced by plain lidocaine for blocks and prilocaine with felypressin for infiltration anesthesia. No significant elevation in ammonia levels was observed. In patients with CTLN1, sedation can be useful for preventing hyperammonemia. Patients who develop symptomatic hyperammonemia may require urgent\/emergent treatment involving other medical specialists. Therefore, preoperative endocrinology consultation, perioperative monitoring of serum ammonia levels, and preemptively coordinating for appropriate care in the event hyperammonemia occurs should all be considered.<\/jats:p>","DOI":"10.2344\/anpr-68-02-04","type":"journal-article","created":{"date-parts":[[2021,10,5]],"date-time":"2021-10-05T16:28:21Z","timestamp":1633451301000},"page":"158-162","source":"Crossref","is-referenced-by-count":0,"title":["<b>Anesthetic Management of a Patient With Citrullinemia Type I During Dental Treatment<\/b>"],"prefix":"10.2344","volume":"68","author":[{"given":"Makiko","family":"Shibuya","sequence":"first","affiliation":[{"name":"Department of Dental Anesthesiology, Faculty of Dental Medicine and Graduate School of Dental Medicine, Hokkaido University, Hokkaido, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rie","family":"Iwamoto","sequence":"additional","affiliation":[{"name":"Department of Dental Anesthesiology, Faculty of Dental Medicine and Graduate School of Dental Medicine, Hokkaido University, Hokkaido, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yukifumi","family":"Kimura","sequence":"additional","affiliation":[{"name":"Department of Dental Anesthesiology, Faculty of Dental Medicine and Graduate School of Dental Medicine, Hokkaido University, Hokkaido, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobuhito","family":"Kamekura","sequence":"additional","affiliation":[{"name":"Department of Dental Anesthesiology, Faculty of Dental Medicine and Graduate School of Dental Medicine, Hokkaido University, Hokkaido, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshiaki","family":"Fujisawa","sequence":"additional","affiliation":[{"name":"Department of Dental Anesthesiology, Faculty of Dental Medicine and Graduate School of Dental Medicine, Hokkaido University, Hokkaido, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1191","published-online":{"date-parts":[[2021,10,4]]},"reference":[{"key":"2022042506263001300_i0003-3006-68-3-158-b01","doi-asserted-by":"crossref","unstructured":"Fukushima\n              K,\n            \n            \n              Yazaki\n              M,\n            \n            \n              Nakamura\n              M,\n            \n            et al.\n\t\t\t\t\t\n          Conventional diet therapy for hyperammonemia is risky in the treatment of hepatic encephalopathy associated with citrin deficiency.\n\t\t\t\t\tIntern Med.\n\t\t\t\t\t2010;\n\t\t\t\t\t49:\n\t\t\t\t\t243\u2013\n\t\t\t\t\t247.","DOI":"10.2169\/internalmedicine.49.2712"},{"key":"2022042506263001300_i0003-3006-68-3-158-b02","doi-asserted-by":"crossref","unstructured":"Nagata\n              N,\n            \n            \n              Matsuda\n              I,\n            \n            \n              Oyanagi\n              K.\n            \n          \n          Estimated frequency of urea cycle enzymopathies in Japan.\n\t\t\t\t\tAm J Med Genet.\n\t\t\t\t\t1991;\n\t\t\t\t\t39:\n\t\t\t\t\t228\u2013\n\t\t\t\t\t229.","DOI":"10.1002\/ajmg.1320390226"},{"key":"2022042506263001300_i0003-3006-68-3-158-b03","doi-asserted-by":"crossref","unstructured":"Gao\n              HZ,\n            \n            \n              Kobayashi\n              K,\n            \n            \n              Tabata\n              A,\n            \n            et al.\n\t\t\t\t\t\n          Identification of 16 novel mutations in the argininosuccinate synthetase gene and genotype-phenotype correlation in 38 classical citrullinemia patients.\n\t\t\t\t\tHum Mutat.\n\t\t\t\t\t2003;\n\t\t\t\t\t22:\n\t\t\t\t\t24\u2013\n\t\t\t\t\t34.","DOI":"10.1002\/humu.10230"},{"key":"2022042506263001300_i0003-3006-68-3-158-b04","unstructured":"Citrullinemia Type 1, National Organization for Rare Disorders.\n\t\t\t\t\tAvailable at: https:\/\/rarediseases.org\/rare-diseases\/citrullinemia-type-1\/. Accessed December 1,2020."},{"key":"2022042506263001300_i0003-3006-68-3-158-b05","doi-asserted-by":"crossref","unstructured":"Choi\n              JJ,\n            \n            \n              Kim\n              HS,\n            \n            \n              Lee\n              KC,\n            \n            \n              Shin\n              Y,\n            \n            \n              Jo\n              YY.\n            \n          \n          Anesthetic experience of an adult male with citrullinemia type II: a case report.\n\t\t\t\t\tBMC Anesthesiol.\n\t\t\t\t\t2016;\n\t\t\t\t\t16:\n\t\t\t\t\t92.","DOI":"10.1186\/s12871-016-0253-7"},{"key":"2022042506263001300_i0003-3006-68-3-158-b06","unstructured":"Igarashi\n              M,\n            \n            \n              Kawana\n              S,\n            \n            \n              Iwasaki\n              H,\n            \n            \n              Namiki\n              A.\n            \n          \n          Anesthetic management for a patient with citrullinemia and liver cirrhosis.\n\t\t\t\t\tJpn J Anesthesiol.\n\t\t\t\t\t1995;\n\t\t\t\t\t44:\n\t\t\t\t\t96\u2013\n\t\t\t\t\t99."},{"key":"2022042506263001300_i0003-3006-68-3-158-b07","doi-asserted-by":"crossref","unstructured":"Takada\n              K,\n            \n            \n              Oda\n              S,\n            \n            \n              Okada\n              M,\n            \n            \n              Iwabuchi\n              M,\n            \n            \n              Kawamae\n              K.\n            \n          \n          Anesthetic management of living related liver transportation for a patient with adult-onset type II citrullinemia.\n\t\t\t\t\tJ Jap Soc Clin Anesth.\n\t\t\t\t\t2009;\n\t\t\t\t\t29:\n\t\t\t\t\t474\u2013\n\t\t\t\t\t479.","DOI":"10.2199\/jjsca.29.474"},{"key":"2022042506263001300_i0003-3006-68-3-158-b08","doi-asserted-by":"crossref","unstructured":"Patel\n              H,\n            \n            \n              Kim\n              J,\n            \n            \n              Huncke\n              TK.\n            \n          \n          General anesthesia in a patient with citrullinemia using Precedex as an adjunct to prevent delayed emergence.\n\t\t\t\t\tJ Clin Anesth.\n\t\t\t\t\t2016;\n\t\t\t\t\t33:\n\t\t\t\t\t403\u2013\n\t\t\t\t\t405.","DOI":"10.1016\/j.jclinane.2016.04.045"},{"key":"2022042506263001300_i0003-3006-68-3-158-b09","doi-asserted-by":"crossref","unstructured":"Gharavifard\n              M,\n            \n            \n              Sabzevari\n              A,\n            \n            \n              Eslami\n              R.\n            \n          \n          Anesthetic management in a child with citrullinemia: a case report.\n\t\t\t\t\tAnesth Pain Med.\n\t\t\t\t\t2014;\n\t\t\t\t\t4:\n\t\t\t\t\te21791.","DOI":"10.5812\/aapm.21791"},{"key":"2022042506263001300_i0003-3006-68-3-158-b10","doi-asserted-by":"crossref","unstructured":"Enns\n              GM,\n            \n            \n              Berry\n              SA,\n            \n            \n              Berry\n              GT,\n            \n            \n              Rhead\n              WJ,\n            \n            \n              Brusilow\n              SW,\n            \n            \n              Hamosh\n              A.\n            \n          \n          Survival after treatment with phenylacetate and benzoate for urea-cycle disorders.\n\t\t\t\t\tN Engl J Med.\n\t\t\t\t\t2007;\n\t\t\t\t\t356:\n\t\t\t\t\t2282\u2013\n\t\t\t\t\t2292.","DOI":"10.1056\/NEJMoa066596"}],"container-title":["Anesthesia Progress"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/meridian.allenpress.com\/anesthesia-progress\/article-pdf\/68\/3\/158\/2930071\/i0003-3006-68-3-158.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/meridian.allenpress.com\/anesthesia-progress\/article-pdf\/68\/3\/158\/2930071\/i0003-3006-68-3-158.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,18]],"date-time":"2024-01-18T19:51:24Z","timestamp":1705607484000},"score":1,"resource":{"primary":{"URL":"https:\/\/anesthesiaprogress.kglmeridian.com\/view\/journals\/anpr\/68\/3\/article-p158.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,1]]},"references-count":10,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2021,10,4]]},"published-print":{"date-parts":[[2021,10,1]]}},"URL":"https:\/\/doi.org\/10.2344\/anpr-68-02-04","relation":{},"ISSN":["1878-7177","0003-3006"],"issn-type":[{"value":"1878-7177","type":"electronic"},{"value":"0003-3006","type":"print"}],"subject":[],"published":{"date-parts":[[2021,10,1]]}}}