{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T16:15:52Z","timestamp":1753892152123,"version":"3.41.2"},"reference-count":40,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T00:00:00Z","timestamp":1671062400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pharmacol."],"abstract":"<jats:p><jats:bold>Background:<\/jats:bold> Protein glycosylation plays an important role in post-translational modification, which defines a broad spectrum of protein functions. Accordingly, infants with a congenital disorder of glycosylation (CDG) can have N-glycosylation, O-glycosylation, or combined N- and O-glycosylation defects, resulting in similar but different multisystem involvement. CDGs can present notable gastrointestinal and neurologic symptoms. Both protein-losing enteropathy and hypotonia affect the decision of using anesthetics. We reported a case of MPI-CDG with protein-losing enteropathy and muscular hypotonia that underwent different anesthesia approach strategies of vascular access. Here, we highlight why intubation with sevoflurane anesthesia and sparing use of muscle relaxants is the optimal strategy for such a condition.<\/jats:p><jats:p><jats:bold>Case presentation:<\/jats:bold> A 25-month-old girl, weighing 6.6\u00a0kg and 64\u00a0cm tall, suffered chronic diarrhea, hypoalbuminemia, and hypotonia since birth. Protein-losing enteropathy due to MPI-CDG was documented by whole-exome sequencing. She underwent three sedated surgical procedures in our hospital. The sedation was administered twice by pediatricians with oral chloral hydrate, intravenous midazolam, and ketamine, to which the patient showed moderate to late recovery from sedation and irritability the following night. The most recent one was administered by an anesthesiologist, where endotracheal intubation was performed with sevoflurane as the main anesthetic. The patient regained consciousness immediately after the operation. She had no complications after all three sedation\/anesthesia interventions and was discharged 7\u00a0days later, uneventful after the third general anesthesia procedure.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> We performed safe anesthetic management in a 25-month-old girl with MPI-CDG using sevoflurane under controlled ventilation. She awoke immediately after the procedure. Due to the disease entity, we suggested bypassing the intravenous route to avoid excess volume for drug administration and that muscle relaxant may not be necessary for endotracheal intubation and patient immobilization when performing procedures under general anesthesia in CDG patients.<\/jats:p>","DOI":"10.3389\/fphar.2022.1038090","type":"journal-article","created":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T09:29:48Z","timestamp":1671096588000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case report: The art of anesthesiology\u2014Approaching a minor procedure in a child with MPI-CDG"],"prefix":"10.3389","volume":"13","author":[{"given":"En-Che","family":"Chang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yu-Hsuan","family":"Chang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yu-Shiun","family":"Tsai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yi-Li","family":"Hung","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Min-Jia","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chih-Shung","family":"Wong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2022,12,15]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1002\/jimd.12024","article-title":"International clinical guidelines for the management of phosphomannomutase 2-congenital disorders of glycosylation: Diagnosis, treatment and follow up","volume":"42","author":"Altassan","year":"2019","journal-title":"J. Inherit. Metab. Dis."},{"key":"B2","doi-asserted-by":"publisher","first-page":"i68","DOI":"10.1093\/bja\/aes393","article-title":"Complications and failure of airway management","volume":"109","author":"Cook","year":"2012","journal-title":"Br. J. Anaesth."},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1211\/CP.2018.20205779","article-title":"Patients are being underdosed: We need new guidance on small-volume drug infusions","volume":"10","author":"Cousins","year":"2018","journal-title":"Clin. Pharm."},{"key":"B4","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1007\/s10545-016-0012-4","article-title":"Liver involvement in congenital disorders of glycosylation (CDG). a systematic review of the literature","volume":"40","author":"da Silva","year":"2017","journal-title":"J. Inherit. Metab. Dis."},{"key":"B5","doi-asserted-by":"publisher","first-page":"681","DOI":"10.1016\/j.accpm.2019.02.004","article-title":"Management of the child\u2019s airway under anaesthesia: The French guidelines","volume":"38","author":"Dadure","year":"2019","journal-title":"Anaesth. Crit. Care Pain Med."},{"key":"B6","doi-asserted-by":"publisher","first-page":"715","DOI":"10.1097\/EJA.0000000000000153","article-title":"Reversal of neuromuscular blockade with sugammadex in patients with myasthenia gravis: A case series of 21 patients and review of the literature","volume":"31","author":"de Boer","year":"2014","journal-title":"Eur. J. Anaesthesiol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"2","DOI":"10.1080\/20961790.2017.1285219","article-title":"Metabolism and metabolomics of ketamine: A toxicological approach","volume":"2","author":"Dinis-Oliveira","year":"2017","journal-title":"Forensic Sci. Res."},{"key":"B8","doi-asserted-by":"publisher","first-page":"760","DOI":"10.1097\/00000542-198811000-00021","article-title":"Resistance to succinylcholine in myasthenia gravis: A dose-response study","volume":"69","author":"Eisenkraft","year":"1988","journal-title":"Anesthesiology"},{"key":"B9","doi-asserted-by":"publisher","first-page":"436","DOI":"10.3389\/fgene.2018.00436","article-title":"Modeling congenital disorders of n-linked glycoprotein glycosylation in drosophila melanogaster","volume":"9","author":"Frappaolo","year":"2018","journal-title":"Front. Genet."},{"key":"B10","doi-asserted-by":"publisher","first-page":"161","DOI":"10.1016\/j.ajhg.2013.10.024","article-title":"Solving glycosylation disorders: Fundamental approaches reveal complicated pathways","volume":"94","author":"Freeze","year":"2014","journal-title":"Am. J. Hum. Genet."},{"key":"B11","doi-asserted-by":"publisher","first-page":"1309","DOI":"10.1542\/peds.107.6.1309","article-title":"Outcome in children receiving continuous venovenous hemofiltration","volume":"107","author":"Goldstein","year":"2001","journal-title":"Pediatrics"},{"key":"B12","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1155\/2015\/985686","article-title":"Flushing and locking of venous catheters: Available evidence and evidence deficit","volume":"2015","author":"Goossens","year":"2015","journal-title":"Nurs. Res. Pract."},{"key":"B13","first-page":"1330","article-title":"Is muscle relaxant necessary for cardiac surgery?","volume-title":"Anesth. Analg.","author":"Gueret","year":"2004"},{"key":"B14","doi-asserted-by":"publisher","first-page":"412","DOI":"10.1016\/S2213-2600(17)30116-9","article-title":"Incidence of severe critical events in paediatric anaesthesia (apricot): A prospective multicentre observational study in 261 hospitals in Europe","volume":"5","author":"Habre","year":"2017","journal-title":"Lancet. Respir. Med."},{"key":"B15","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1097\/NAN.0000000000000355","article-title":"Best practice for delivering small-volume intermittent intravenous infusions","volume":"43","author":"Harding","year":"2020","journal-title":"J. Infus. Nurs."},{"key":"B16","doi-asserted-by":"publisher","first-page":"823","DOI":"10.1542\/peds.19.5.823","article-title":"The maintenance need for water in parenteral fluid therapy","volume":"19","author":"Holliday","year":"1957","journal-title":"Pediatrics"},{"key":"B17","doi-asserted-by":"publisher","first-page":"1000","DOI":"10.1007\/s12630-015-0423-y","article-title":"Managing the challenging pediatric airway: Continuing professional development","volume":"62","author":"Karsli","year":"2015","journal-title":"Can. J. Anaesth."},{"volume-title":"Nelson textbook of pediatrics","year":"2016","author":"Kliegman","key":"B18"},{"key":"B19","doi-asserted-by":"publisher","first-page":"283","DOI":"10.4103\/0259-1162.143110","article-title":"Ketamine: Current applications in anesthesia, pain, and critical care","volume":"8","author":"Kurdi","year":"2014","journal-title":"Anesth. Essays Res."},{"key":"B20","doi-asserted-by":"publisher","first-page":"325","DOI":"10.4236\/ijcm.2011.23056","article-title":"Anesthetic management of a boy with congenital disorder of glycosylation (CDG) i-x","volume":"2","author":"Lehavi","year":"2011","journal-title":"Int. J. Clin. Med."},{"key":"B21","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1111\/j.1460-9592.2005.01671.x","article-title":"Anesthesia considerations in carbohydrate-deficient glycoprotein syndrome type i","volume":"15","author":"Meaudre","year":"2005","journal-title":"Paediatr. Anaesth."},{"volume-title":"Miller\u2019s anesthesia","year":"2009","author":"Miller","key":"B22"},{"key":"B23","doi-asserted-by":"publisher","first-page":"337","DOI":"10.1093\/bja\/ael368","article-title":"Neuromuscular effects of sevoflurane in myasthenia gravis patients","volume":"98","author":"Nitahara","year":"2007","journal-title":"Br. J. Anaesth."},{"key":"B24","doi-asserted-by":"publisher","first-page":"629","DOI":"10.1016\/j.jpedsurg.2012.08.018","article-title":"Oral ketamine and midazolam for pediatric burn patients: A prospective, randomized, double-blind study","volume":"48","author":"Norambuena","year":"2013","journal-title":"J. Pediatr. Surg."},{"key":"B25","doi-asserted-by":"publisher","first-page":"172547","DOI":"10.1016\/j.ejphar.2019.172547","article-title":"Ketamine applications beyond anesthesia \u2013 A literature review","volume":"860","author":"Nowacka","year":"2019","journal-title":"Eur. J. Pharmacol."},{"key":"B26","article-title":"Ketamine toxicity","volume-title":"StatPearls","author":"Orhurhu1","year":"2021"},{"key":"B27","article-title":"Specific considerations with liver disease - metabolism of anesthetic","volume-title":"Clinical anesthesia procedures of the Massachusetts general hospital","author":"Pino","year":"2019"},{"key":"B28","doi-asserted-by":"publisher","first-page":"547","DOI":"10.1007\/BF03018638","article-title":"Propofol or sevoflurane anesthesia without muscle relaxants allow the early extubation of myasthenic patients","volume":"50","author":"Rocca","year":"2003","journal-title":"Can. J. Anaesth."},{"key":"B29","article-title":"Ketamine","volume-title":"StatPearls","author":"Rosenbaum","year":"2021"},{"key":"B30","doi-asserted-by":"publisher","first-page":"01","DOI":"10.1007\/s12105-021-01301-z","article-title":"Craniofacial development: Neural crest in molecular embryology","volume":"15","author":"Roth","year":"2021","journal-title":"Head. Neck Pathol."},{"key":"B31","doi-asserted-by":"publisher","first-page":"S6-S8","DOI":"10.12968\/bjon.2020.29.2.S6","article-title":"Residual fluid after IV infusion drug administration: Risk of suboptimal dosing","volume":"29","author":"Rout","year":"2020","journal-title":"Br. J. Nurs."},{"key":"B32","doi-asserted-by":"publisher","first-page":"e01180","DOI":"10.1213\/XAA.0000000000001180","article-title":"Hypoalbuminemia-related prolonged sedation after general anesthesia: A case report","volume":"14","author":"Saad","year":"2020","journal-title":"A. A. Pract."},{"key":"B33","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1186\/s40981-017-0080-y","article-title":"Anesthetic management of a child with phosphomannomutase-2 congenital disorder of glycosylation (pmm2-cdg)","volume":"3","author":"Sakai","year":"2017","journal-title":"JA Clin. Rep."},{"key":"B34","doi-asserted-by":"publisher","first-page":"550","DOI":"10.1136\/jmg.2003.016923","article-title":"Clinical and molecular features of three patients with congenital disorders of glycosylation type ih (CDG-Ih) (ALG8 deficiency)","volume":"41","author":"Schollen","year":"2004","journal-title":"J. Med. Genet."},{"key":"B35","doi-asserted-by":"publisher","first-page":"729","DOI":"10.1097\/ALN.0b013e318280a40f","article-title":"Situation awareness in anesthesia: Concept and research","volume":"118","author":"Schulz","year":"2013","journal-title":"Anesthesiology"},{"key":"B36","doi-asserted-by":"publisher","first-page":"1166","DOI":"10.1007\/s00134-011-2231-3","article-title":"Weight-based determination of fluid overload status and mortality in pediatric intensive care unit patients requiring continuous renal replacement therapy","volume":"37","author":"Selewski","year":"2011","journal-title":"Intensive Care Med."},{"key":"B37","doi-asserted-by":"publisher","first-page":"379","DOI":"10.1213\/01.ANE.0000131964.67524.E7","article-title":"Capnography accurately detects apnea during monitored anesthesia care","volume":"379","author":"Soto","year":"2004","journal-title":"Anesth. Analg."},{"key":"B38","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1111\/pan.13050","article-title":"Current evidence for the use of sugammadex in children","volume":"27","author":"Tobias","year":"2017","journal-title":"Paediatr. Anaesth."},{"volume-title":"Katzung & trevor\u2019s pharmacology examination and board review","year":"2019","author":"Trevor","key":"B39"},{"key":"B40","doi-asserted-by":"publisher","first-page":"850","DOI":"10.1097\/EJA.0000000000001500","article-title":"Rocuronium reversed with sugammadex for thymectomy in myasthenia gravis: A retrospective analysis of complications from Japan","volume":"38","author":"Tsukada","year":"2021","journal-title":"Eur. J. Anaesthesiol."}],"container-title":["Frontiers in Pharmacology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fphar.2022.1038090\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T09:29:54Z","timestamp":1671096594000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fphar.2022.1038090\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,15]]},"references-count":40,"alternative-id":["10.3389\/fphar.2022.1038090"],"URL":"https:\/\/doi.org\/10.3389\/fphar.2022.1038090","relation":{},"ISSN":["1663-9812"],"issn-type":[{"type":"electronic","value":"1663-9812"}],"subject":[],"published":{"date-parts":[[2022,12,15]]},"article-number":"1038090"}}