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BMC Anesthesiology

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Open Access 01-12-2016 | Case report

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

Authors: Jung Ju Choi, Hong Soon Kim, Kyung Cheon Lee, Youseok Shin, Youn Yi Jo

Published in: BMC Anesthesiology | Issue 1/2015

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.

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Title: Anesthetic experience of an adult male with citrullinemia type II: a case report
Authors: Jung Ju Choi
Hong Soon Kim
Kyung Cheon Lee
Youseok Shin
Youn Yi Jo
Publication date: 01-12-2016
Publisher: BioMed Central
Published in: BMC Anesthesiology / Issue 1/2015
Electronic ISSN: 1471-2253
DOI: https://doi.org/10.1186/s12871-016-0253-7

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

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

Abstract

Background

Case presentation

Conclusion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Case presentation

Conclusion

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