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Chapter 4 - Acute Liver Failure in Children

from Section I - Pathophysiology of Pediatric Liver Disease

Published online by Cambridge University Press:  19 January 2021

By
James Squires  and
Estella Alonso
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
Edited in association with
Jorge A. Bezerra ,
Cara L. Mack  and
Benjamin L. Shneider
Frederick J. Suchy
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Ronald J. Sokol
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
William F. Balistreri
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Jorge A. Bezerra
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Cara L. Mack
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Benjamin L. Shneider
Affiliation:
Texas Children’s Hospital, Houston
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Summary

Pediatric acute liver failure (PALF) is not a single diagnosis. Rather, PALF is a complex, rapidly progressive clinical syndrome that is the final common pathway for many disparate conditions; some known and others yet to be identified [1, 2]. The estimated frequency of acute liver failure (ALF) in all age groups in the USA is about 17 cases per 100,000 population per year, but the frequency in children is unknown. In the USA, ALF accounts for 10–15% of pediatric liver transplants performed annually [3]. Management requires a multidisciplinary team involving the hepatologist, critical care specialist, and liver transplant surgeon. Acute liver failure is a rapidly evolving clinical condition. The absence of adequately powered studies to inform diagnostic algorithms, to assess markers of disease severity and trajectory, and to guide liver transplant decisions transfers a significant burden to the clinician. Constructing a diagnostic approach and individualized management strategy that may include the decision to pursue liver transplantation is challenging. There are a number of pressing clinical questions faced when children with PALF first present. Does the patient have a condition that is treatable? What is the risk of deterioration or improvement on each day the child is alive with his/her native liver? Is a living related or deceased liver transplant necessary for patient survival? Is full recovery possible without a liver transplant? Are associated morbidities recoverable or irreversible?

Type
Chapter
Information
Liver Disease in Children , pp. 36 - 57
Publisher: Cambridge University Press
Print publication year: 2021

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