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Case Reports
. 2016 Oct 11;16(1):92.
doi: 10.1186/s12871-016-0253-7.

Anesthetic experience of an adult male with citrullinemia type II: a case report

Jung Ju Choi  1 , Hong Soon Kim  1 , Kyung Cheon Lee  1 , Youseok Shin  1 , Youn Yi Jo  2
Affiliations
Case Reports

Anesthetic experience of an adult male with citrullinemia type II: a case report

Jung Ju Choi et al. BMC Anesthesiol. .

Abstract

Background: Citrullinemia type II is an autosomal recessive urea cycle disorder and a subtype of citrin deficiency. However, the management of recurrent hyperammonemia with neurologic symptoms in patients with citrullinemia type II is quite different from the management of other types of urea cycle disorders. In pats with citrullinemia type II, regional anesthesia might be a good choice for the early detection of hyperammonemic symptoms and addressing psychic stress.

Case presentation: A 48-year-old male with adult onset citrullinemia type II was scheduled for urethral scrotal fistula repair. During the first operation, spinal anesthesia with conscious sedation using dexmedetomidine was used, a second operation was performed after confirmation of infection control and a stable neurologic condition. In this patient, dietary planning with close monitoring of serum ammonia level and close observation of neurologic conditions might lead to successful perioperative care.

Conclusion: For anesthesia of patients with adult onset citrullinemia type II, close monitoring of neurologic signs and serum ammonia are important to reduce neurologic complications induced by hyperammonemia. Regional anesthesia with a proper dietary plan might reduce patient stress and prevent metabolic tragedy.

Keywords: Adult onset citrullinemia type II; Anesthesia; Hyperammonemia.

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Figures

Fig. 1
Fig. 1
Perioperative serum ammonia level changes
See this image and copyright information in PMC

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