Pediatric Fulminant Hepatic Failure Workup

Updated: Aug 09, 2017
  • Author: Hisham Nazer, MBBCh, FRCP, DTM&H; Chief Editor: Carmen Cuffari, MD  more...
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Workup

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.

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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.

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Metabolic Panel

Glucose level is decreased, especially in infants. Hyponatremia (see Serum Sodium), hyperkalemia (see Potassium), respiratory alkalosis, or metabolic acidosis (see Acid-Base Interpretation) may also be present.

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Renal Function Studies

Serum creatinine, phosphate, and other levels have been recognized recently as strong predictors of survival and the need for transplantation.

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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.

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Tests for Viral Causes

Hepatitis A virus (HAV), hepatitis B virus (HBV; see Hepatitis B Test), hepatitis C virus (HCV; see Hepatitis C Test), hepatitis D virus (HDV), hepatitis E virus and many other 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.

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Liver Biopsy

Liver biopsy may contribute to the working diagnosis and subsequent therapy. However, samples should be examined with caution because results correlate poorly with prognosis. In view of the presence of coagulopathy, weigh the risk of liver biopsy against its contribution to diagnosis and management.

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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