Acute Liver Failure, Inborn Errors of Metabolism and Diabetic Ketoacidosis

9.10 - Acute Liver Failure, Inborn Errors of Metabolism and Diabetic Ketoacidosis

from Section 9 - Paediatric Care

Published online by Cambridge University Press: 27 July 2023

Andrew J. Jones and

Edited by

Debashish Dutta and

Ned Gilbert-Kawai: Affiliation:
The Royal Liverpool Hospital
Debashish Dutta: Affiliation:
Princess Alexandra Hospital NHS Trust, Harlow
Carl Waldmann: Affiliation:
Royal Berkshire Hospital, Reading

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Summary

Key Learning Points

1. Children with acute liver failure (ALF) require rapid stabilisation and should be moved to a specialist centre as quickly as possible.
2. Once on a paediatric intensive care unit, ALF patients undergo standard neurocritical intensive care and a transplant assessment.
3. In ALF, children with encephalopathy more severe than grade 1 should be considered for intubation.
4. The mean arterial pressures targeted for encephalopathic patients vary with age.
5. In diabetic ketoacidosis, cerebral oedema is the most common cause of mortality and neurological observations should be performed regularly.

Keywords

acute liver failure hepatic encephalopathy transplant metabolism diabetic ketoacidosis

Type: Chapter
Information: Intensive Care Medicine
The Essential Guide
, pp. 722 - 725

DOI: https://doi.org/10.1017/9781108989060 [Opens in a new window]

Publisher: Cambridge University Press

Print publication year: 2021

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References

References and Further Reading

British Inherited Metabolic Diseases Group (BIMDG). bimdg.org.uk/site/index.asp Google Scholar

British Inherited Metabolic Diseases Group (BIMDG). Emergency guidelines. bimdg.org.uk/site/guidelines.asp Google Scholar

British Society for Paediatric Endocrinology and Diabetes. Guidelines. www.bsped.org.uk/clinical-resources/guidelines/Google Scholar

British Society for Paediatric Endocrinology and Diabetes. BSPED interim guideline for the management of children and young people under the age of 18 years with diabetic ketoacidosis. www.bsped.org.uk/clinical/docs/DKAGuideline.pdf Google Scholar

Champion, M. An approach to the diagnosis of inherited metabolic disease. Arch Dis Child Educ Pract 2010;95:40–6.Google Scholar

Conn, HO. Quantifying the severity of hepatic encephalopathy. In: Conn, HO, Bircher, J (eds). Hepatic Encephalopathy: Syndromes and Therapies. Bloomington, IL: Medi-ed Press; 1994. pp. 13–26.Google Scholar

Durand, P, Debray, D, Mandel, R, et al. Acute liver failure in infancy: a 14-year experience of a pediatric liver transplantation center. J Pediatr 2001;139:871–6.Google Scholar

Kuppermann, N, Ghetti, S, Schunk, JE, et al. Clinical trial of fluid infusion rates for pediatric diabetic ketoacidosis. N Engl J Med 2018;378:2275–87.Google Scholar

Lee, WM, Stravitz, RT, Larson, AM. Introduction to the revised American Association for the Study of Liver Diseases Position Paper on acute liver failure 2011. Hepatology 2012;55:965–7.CrossRef Google Scholar

Squires, RH Jr, Shneider, BL, Bucuvalas, J, et al. Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group. J Pediatr 2006;148:652–8.CrossRef Google Scholar PubMed

Wolfdorsf, JI. The International Society of Pediatric and Adolescent Diabetes guidelines for management of diabetic ketoacidosis: do the guidelines need to be modified? Pediatr Diabetes 2014:15:277–86.Google Scholar