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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

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. 1. Children with acute liver failure (ALF) require rapid stabilisation and should be moved to a specialist centre as quickly as possible.

  2. 2. Once on a paediatric intensive care unit, ALF patients undergo standard neurocritical intensive care and a transplant assessment.

  3. 3. In ALF, children with encephalopathy more severe than grade 1 should be considered for intubation.

  4. 4. The mean arterial pressures targeted for encephalopathic patients vary with age.

  5. 5. In diabetic ketoacidosis, cerebral oedema is the most common cause of mortality and neurological observations should be performed regularly.

Type
Chapter
Information
Intensive Care Medicine
The Essential Guide
, pp. 722 - 725
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.aspGoogle Scholar
British Inherited Metabolic Diseases Group (BIMDG). Emergency guidelines. bimdg.org.uk/site/guidelines.aspGoogle 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.pdfGoogle 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. 1326.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.CrossRefGoogle 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.CrossRefGoogle ScholarPubMed
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

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