Pediatric Fulminant Hepatic Failure Workup
- Author: Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H; Chief Editor: Carmen Cuffari, MD more...
Approach Considerations
A range of laboratory studies is required to determine the etiology, severity, and prognosis in patients with fulminant hepatic failure (FHF). Liver biopsy is usually an essential procedure to consider in the management of FHF.
Liver Function Studies
Hepatic enzyme levels do not correlate well with the severity of the disease; they may be elevated, normal, or even decreased in patients with FHF. Levels are often markedly elevated in patients with metabolic disorders. With progressive necrosis of the liver, hepatic enzyme levels decrease.
Both direct and indirect serum bilirubin levels are usually elevated. Typically, conjugated hyperbilirubinemia is present.
Metabolic Panel
Glucose level is decreased, especially in infants. Hyponatremia, hyperkalemia, respiratory alkalosis, or metabolic acidosis may also be present.
Renal Function Studies
Serum creatinine, phosphate, and other levels have been recognized recently as strong predictors of survival and the need for transplantation.
Coagulation Profile
Prothrombin time (PT) is prolonged. However, administration of vitamin K typically has not been found to result in a satisfactory drop in prothrombin time (PT) in patients with FHF.
Tests for Viral Causes
Hepatitis A virus (HAV), hepatitis B virus (HBV), hepatitis C virus (HCV), hepatitis D virus (HDV), and hepatitis E virus account for approximately 50% of cases. Many viruses other than hepatitis also are recognized causes of FHF in childhood.
HBV is the most common cause of FHF in endemic areas. Presence of immunoglobulin M (IgM) antibody to HBV core antigen (IgM anti-HBcAg) in serum supports the diagnosis of acute HBV infection.
HAV infection is a recognized cause of FHF in individuals of all ages. Diagnosis of HAV infection is made by the presence of anti-HAV IgM in the patient’s serum. HCV infection is diagnosed with detection of anti-HCV antibody or HCV RNA in the serum. HDV is diagnosed by the presence of anti-HDV RNA in the serum.
Other causative viruses include Epstein-Barr virus, cytomegalovirus (CMV), herpesviruses, and adenoviruses.
Liver Biopsy
Liver biopsy contributes to the working diagnosis and subsequent therapy. However, samples should be examined with caution because results correlate poorly with prognosis. Liver biopsy is mostly required to further assist in reaching a likely diagnosis or in preparation for liver transplantation.
In view of the presence of coagulopathy, weigh the risk of liver biopsy against its contribution to diagnosis and management. Transvenous biopsy is not uncommonly used as a relatively safe route in such a clinical situation.
Histologic Findings
Two types of histology have been recognized in patients with FHF. The first type is usually observed in cases that stem from drug reactions or viral hepatitis. This type is characterized by extensive necrosis of the peripheral hepatocytes, with little or no regeneration. Hepatocyte necrosis with microvascular fat accumulation may be observed, especially in patients with FHF secondary to inborn errors of metabolism.
The second type of histology, observed in valproate toxicity, Reye syndrome, and other metabolic liver disease, is characterized by microvesicular steatosis and centrilobular necrosis.
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