Case report: The art of anesthesiology—Approaching a minor procedure in a child with MPI-CDG
Background: 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 defe...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-12-01
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| Series: | Frontiers in Pharmacology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fphar.2022.1038090/full |
| _version_ | 1811295763497484288 |
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| author | En-Che Chang Yu-Hsuan Chang Yu-Shiun Tsai Yi-Li Hung Min-Jia Li Chih-Shung Wong Chih-Shung Wong Chih-Shung Wong |
| author_facet | En-Che Chang Yu-Hsuan Chang Yu-Shiun Tsai Yi-Li Hung Min-Jia Li Chih-Shung Wong Chih-Shung Wong Chih-Shung Wong |
| author_sort | En-Che Chang |
| collection | DOAJ |
| description | Background: 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.Case presentation: A 25-month-old girl, weighing 6.6 kg and 64 cm 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 days later, uneventful after the third general anesthesia procedure.Conclusion: 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. |
| first_indexed | 2024-04-13T05:38:22Z |
| format | Article |
| id | doaj.art-4403370b88a74f8dbddfcaa7e4656a13 |
| institution | Directory Open Access Journal |
| issn | 1663-9812 |
| language | English |
| last_indexed | 2024-04-13T05:38:22Z |
| publishDate | 2022-12-01 |
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| series | Frontiers in Pharmacology |
| spelling | doaj.art-4403370b88a74f8dbddfcaa7e4656a132022-12-22T03:00:12ZengFrontiers Media S.A.Frontiers in Pharmacology1663-98122022-12-011310.3389/fphar.2022.10380901038090Case report: The art of anesthesiology—Approaching a minor procedure in a child with MPI-CDGEn-Che Chang0Yu-Hsuan Chang1Yu-Shiun Tsai2Yi-Li Hung3Min-Jia Li4Chih-Shung Wong5Chih-Shung Wong6Chih-Shung Wong7School of Medicine, Fu-Jen Catholic University, New Taipei, TaiwanSchool of Medicine, Fu-Jen Catholic University, New Taipei, TaiwanSchool of Medicine, Fu-Jen Catholic University, New Taipei, TaiwanDepartment of Pediatrics, Cathay General Hospital, Taipei, TaiwanDepartment of Anesthesiology, Cathay General Hospital, Taipei, TaiwanSchool of Medicine, Fu-Jen Catholic University, New Taipei, TaiwanDepartment of Anesthesiology, Cathay General Hospital, Taipei, TaiwanGraduate Institute of Medical Science, National Defense Medical, Taipei, TaiwanBackground: 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.Case presentation: A 25-month-old girl, weighing 6.6 kg and 64 cm 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 days later, uneventful after the third general anesthesia procedure.Conclusion: 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.https://www.frontiersin.org/articles/10.3389/fphar.2022.1038090/fullCDGketaminesevofluranehypoalbuminemianeuromuscular blocking agentshypotonia |
| spellingShingle | En-Che Chang Yu-Hsuan Chang Yu-Shiun Tsai Yi-Li Hung Min-Jia Li Chih-Shung Wong Chih-Shung Wong Chih-Shung Wong Case report: The art of anesthesiology—Approaching a minor procedure in a child with MPI-CDG Frontiers in Pharmacology CDG ketamine sevoflurane hypoalbuminemia neuromuscular blocking agents hypotonia |
| title | Case report: The art of anesthesiology—Approaching a minor procedure in a child with MPI-CDG |
| title_full | Case report: The art of anesthesiology—Approaching a minor procedure in a child with MPI-CDG |
| title_fullStr | Case report: The art of anesthesiology—Approaching a minor procedure in a child with MPI-CDG |
| title_full_unstemmed | Case report: The art of anesthesiology—Approaching a minor procedure in a child with MPI-CDG |
| title_short | Case report: The art of anesthesiology—Approaching a minor procedure in a child with MPI-CDG |
| title_sort | case report the art of anesthesiology approaching a minor procedure in a child with mpi cdg |
| topic | CDG ketamine sevoflurane hypoalbuminemia neuromuscular blocking agents hypotonia |
| url | https://www.frontiersin.org/articles/10.3389/fphar.2022.1038090/full |
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