Pediatric anesthesia & critical care

Anesthesia management protocol for liver transplantation as treatment for ornithine transcarbamylase deficiency

Paediatr AnaesthPublished Retrospective case series

Authors' conclusion

Liver transplantation for cases with uncontrolled hyperammonemia can be performed with proper management. Second, liver transplantation with carrier donors should be avoided because of the risk of postoperative recurrence.

Study context

Population
Liver transplantation cases for ornithine transcarbamylase deficiency, November 2005–March 2021
Participants
29 liver transplantation cases
Outcome
Perioperative course
Methodological detailssingle-centre
  • Single-centre

Main finding

As reported in the abstract

Stable through the perioperative period

25

Hyperammonemia after liver transplantation, carrier donor graft

2

Uncontrolled hyperammonemia before liver transplantation despite continuous hemodialysis; life-saving transplantation, metabolic status stabilised after anhepatic phase

2

Observational data: associations, not effects.

Original publication

Anesthesia management protocol for liver transplantation as treatment for ornithine transcarbamylase deficiency. Paediatr Anaesth. Published 4 May 2023. doi:10.1111/pan.14691

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