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Glycaemic management in a child with ornithine transcarbamylase deficiency undergoing cardiac surgery with hypothermic cardiopulmonary bypass

Glycaemic management in a child with ornithine transcarbamylase deficiency undergoing cardiac... There is a lack of evidence regarding the optimal intra‐operative glycaemic level of patients with ornithine transcarbamylase deficiency to prevent cerebral oedema due to protein catabolism and hyperammonemia. We describe a case of a two‐year‐old girl with ornithine transcarbamylase deficiency who underwent cardiac surgery requiring cardiopulmonary bypass. A high‐dose dextrose infusion to prevent protein catabolism was given throughout surgery, which caused uncontrollable hyperglycaemia unresponsive to high‐dose insulin administration. Factors contributing to the hyperglycaemia may have included surgical stress, steroid administration and hypothermia. During invasive surgery, anaesthetists should carefully adjust the rates of dextrose and insulin infusions, guided by close monitoring of blood ammonia, glucose and lactate. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Anaesthesia Reports Wiley

Glycaemic management in a child with ornithine transcarbamylase deficiency undergoing cardiac surgery with hypothermic cardiopulmonary bypass

Yamauchi, Y.; Yamada, A.; Kojima, T.
Anaesthesia Reports , Volume 10 (2) – Jul 1, 2022
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Publisher
Wiley
Copyright
2022 © Association of Anaesthetists
eISSN
2637-3726
DOI
10.1002/anr3.12179
Publisher site
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Abstract

There is a lack of evidence regarding the optimal intra‐operative glycaemic level of patients with ornithine transcarbamylase deficiency to prevent cerebral oedema due to protein catabolism and hyperammonemia. We describe a case of a two‐year‐old girl with ornithine transcarbamylase deficiency who underwent cardiac surgery requiring cardiopulmonary bypass. A high‐dose dextrose infusion to prevent protein catabolism was given throughout surgery, which caused uncontrollable hyperglycaemia unresponsive to high‐dose insulin administration. Factors contributing to the hyperglycaemia may have included surgical stress, steroid administration and hypothermia. During invasive surgery, anaesthetists should carefully adjust the rates of dextrose and insulin infusions, guided by close monitoring of blood ammonia, glucose and lactate.

Journal

Anaesthesia Reports – Wiley

Published: Jul 1, 2022

Keywords: cardiopulmonary bypass; cerebral oedema; hyperammonemia; hyperglycaemia; hypothermia; ornithine transcarbamylase deficiency

References

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    Chiong, MA; Bennetts, BH; Strasser, SI; Wilcken, B
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    Bruns, N; Große Lordemann, A; Rasche, T
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    Polderman, KH
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    Gibbison, B; Villalobos Lizardi, JC; Avilés Martínez, KI
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