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Pediatric Fulminant Hepatic Failure Treatment & Management

  • Author: Hisham Nazer, MB, BCh, FRCP, , DTM&H; Chief Editor: Carmen Cuffari, MD  more...
 
Updated: Aug 18, 2015
 

Approach Considerations

Reaching a diagnosis of fulminant hepatic failure (FHF) is of vital importance so that appropriate and early treatment can be initiated. Unfortunately, in most patients, no definitive therapy that can result in regeneration of hepatocytes or reversal of injury is available.[18]

Provide symptomatic treatment and life support. Direct treatment toward the specific cause of FHF when an identifiable etiology is found. Avoid nephrotoxic agents, benzodiazepines, and other sedative medications. An intensive care unit (ICU) and pediatric hepatology setting with facilities for liver transplantation should be available for proper diagnosis and management.

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General Supportive Care

General supportive care includes correction of any fluid and electrolyte imbalances and management of hypoglycemia if present.

Correction of fluid and electrolyte abnormalities

Avoid fluid overload (restrict hydration up to 2 mL/kg/h). Hemodynamic monitoring of central pressures is advised to assess volume depletion and overload.

Monitor electrolytes and correct any disturbances. Patients may require intravenous administration of calcium, phosphorus, or magnesium.

Management of hypoglycemia

Monitor blood glucose regularly for possible complicating hypoglycemia, and treat with intravenous glucose administration. An infusion of 10-20% of glucose is usually required.

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Correction of Coagulopathy

Parenteral vitamin K and plasmapheresis are needed to correct coagulopathy and prevent its serious sequelae. However, unless acute hemorrhage is present or an invasive procedure is performed, empiric transfusion with fresh frozen plasma (FFP) is not warranted. It can present a significant volume challenge to the kidneys.

Platelet transfusion may be indicated in severe cases of FHF with coagulopathy and thrombocytopenia. It occasionally is required to maintain a platelet count of greater than 50,000.

A parenteral H2 -receptor blocker is administered prophylactically to prevent potential gastrointestinal (GI) bleeding.

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Treatment of Specific Causes of Fulminant Hepatic Failure

Hepatitis is treated with acyclovir for herpesvirus hepatitis and with prednisone and azathioprine for autoimmune hepatitis.

Acetaminophen overdose is treated with an antidote for hepatotoxicity (ie, N -acetylcysteine).

Galactosemia and fructosemia are treated with dietary elimination. Hereditary tyrosinemia type I is treated with dietary elimination and 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC).

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Management of Renal Dysfunction

Renal dysfunction with renal failure occurs in as many as 50% of patients. Kidneys are involved secondary to hepatorenal syndrome (HRS), acute tubular necrosis (ATN), drug-induced nephrotoxicity, or prerenal azotemia. Therefore, monitoring fluids and renal function tests is important.

Maintain urine output. Focus on management of renal impairment due to HRS or ATN.

Management of hepatorenal syndrome

HRS is defined as functional renal failure occurring in patients with severe liver disease in the absence of any other underlying cause of renal disease. A decrease in blood flow to the kidneys has been suggested as the underlying pathophysiology.

Pay special attention to risk factors leading to development of HRS, including low sodium and high potassium levels in the serum, low plasma osmolarity, high urine osmolarity, and poor nutritional status. Avoid large-volume paracentesis without plasma volume replacement.[19]

Liver transplantation is the treatment of choice for HRS; however, some patients continue to require dialysis following the transplant.

Peritoneal dialysis, hemodialysis, and hemofiltration have limited benefit and, thus, remain controversial in HRS. Systemic vasoconstricting agents and renal vasodilators are used but have limited value. Medical therapy is considered a temporary measure to improve renal function while the child with HRS is waiting for liver transplantation. Vasoconstrictors, such as the vasopressin analog terlipressin, have shown promising results in adult patients with HRS. Terlipressin administration results in splanchnic vasoconstriction and thus an increase in systemic and renal perfusion in HRS. Therapy with terlipressin constitutes a bridge towards liver transplantation and improves the prognosis after transplantation.

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Management of Cerebral Edema

Cerebral edema occurs in as many as 80% of patients. It increases intracranial pressure (ICP), resulting in impaired cerebral effusion. This can result in irreversible neurologic damage, and death. Cytotoxic and vasogenic edema are present, presumably caused by release of neurotoxins in the circulation.

Insertion of an ICP monitor in patients with grade 3 encephalopathy is advisable to detect cerebral edema early in its course.

Preventive measures include positioning the patient with the head elevated and avoidance of any manipulations that increase ICP. Other preventive measures are the avoidance of hypothermia and hypercapnia, controlling agitation, and instituting moderate hyperventilation.

Continuously monitoring ICP in severe illness is of vital importance, especially in stage 3 or 4 of hepatic encephalopathy. Mannitol is used in patients with documented ICP greater than 30 mm Hg and is considered in patients with progressive edema.

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Treatment of Nonviral Infections

Bacterial and fungal infections commonly occur. Use appropriate antibiotics to treat serious infections, septicemia, peritonitis, urinary tract infections, and pneumonia.

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Bowel Management

Use lactulose enemas to evacuate the bowel. Oral neomycin is indicated to decrease enteric bacteria that produce ammonia.

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Liver Transplantation

Orthotopic liver transplantation (OLT) remains the only effective mode of treatment of FHF. FHF is the indication for 11-13% of liver transplantations and carries an important prognostic implication. Consider OLT in any patient presenting with FHF, regardless of the etiology.[20] Consider urgent transplant when the international normalized ratio (INR) reaches 4, especially in very young children.[21, 22]

A more recent approach is to try using liver-assist devices, such as matrices of cultured hepatocytes, to support the patient’s liver until hepatic regeneration occurs or a suitable donor is made available for liver transplantation.[23, 24]

In an acute emergency, segment liver transplant or living related donor transplant is performed to spare the child with FHF the potentially fatal outcome of rapidly progressive liver necrosis. Innovative approaches, such as auxiliary hepatic transplantation,[25, 26] xenograft, extracorporeal human liver, and artificial liver support devices, also are considered in emergency situations. 

Despite technical difficulties and a donor organ shortage, the results of OLT in the pediatric age group with end-stage liver disease have demonstrated promising results. Therefore, early referral to a specialized center for liver transplantation is vital.[24, 6]

Go to Pediatric Liver Transplantation and Liver Transplants for more complete information on these topics.

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Dietary Measures

Special attention to diet is indicated. Patients require high calories, high carbohydrates, and moderate fat. Total parenteral nutrition (TPN) may be needed to ensure adequate nutrition, especially when enteral feeding is not possible. Special formulas are available that are high in branched-chain amino acids and low in aromatic amino acids and electrolytes.

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Consultations

Consultations with the following specialists may be indicated:

  • Gastroenterologist
  • Neurosurgeon
  • Hematologist
  • Infectious disease specialist
  • Transplantation surgeon
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Contributor Information and Disclosures
Author

Hisham Nazer, MB, BCh, FRCP, , DTM&H Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, University of Jordan Faculty of Medicine, Jordan

Hisham Nazer, MB, BCh, FRCP, , DTM&H is a member of the following medical societies: American Association for Physician Leadership, Royal College of Paediatrics and Child Health, Royal College of Surgeons in Ireland, Royal Society of Tropical Medicine and Hygiene, Royal College of Physicians and Surgeons of the United Kingdom

Disclosure: Nothing to disclose.

Coauthor(s)

Dena Nazer, MD, FAAP Assistant Professor of Pediatrics, Wayne State University School of Medicine; Chief, Child Protection Team, Children's Hospital of Michigan

Dena Nazer, MD, FAAP is a member of the following medical societies: Academic Pediatric Association, American Academy of Pediatrics, American Professional Society on the Abuse of Children, Ray E Helfer Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching.

Additional Contributors

Jayant Deodhar, MD Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India

Disclosure: Nothing to disclose.

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