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Anesthetic Management of a Patient With Citrullinemia Type I During Dental Treatment.

Authors
  • Shibuya, Makiko1
  • Iwamoto, Rie1
  • Kimura, Yukifumi1
  • Kamekura, Nobuhito1
  • Fujisawa, Toshiaki1
  • 1 Department of Dental Anesthesiology, Faculty of Dental Medicine and Graduate School of Dental Medicine, Hokkaido University, Hokkaido, Japan. , (Japan)
Type
Published Article
Journal
Anesthesia progress
Publication Date
Oct 01, 2021
Volume
68
Issue
3
Pages
158–162
Identifiers
DOI: 10.2344/anpr-68-02-04
PMID: 34606567
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

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. © 2021 by the American Dental Society of Anesthesiology.

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