eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Fulminant Hepatic Failure: Follow-up

Author: Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H, Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, Bushnaq Medical Centre, University of Jordan
Coauthor(s): Dena Nazer, MD, Fellow, Child Protection Center, Children's Hospital of Michigan
Contributor Information and Disclosures

Updated: Nov 12, 2008

Follow-up

Further Inpatient Care

Further inpatient care in patients with fulminant hepatic failure (FHF) is directed towards prevention and treatment of developing complications. This includes coagulation abnormalities, immune deficiencies, encephalopathy, and cerebral edema. Early referral to a liver transplantation center is recommended.

Transfer

An ICU and pediatric hepatology setting with facilities for liver transplantation should be available for proper diagnosis and management.

Deterrence/Prevention

Preventive methods to avoid cerebral edema and renal failure should be performed.

Complications

  • Infections: Bacterial and fungal infections commonly occur, leading to the development of peritonitis, pneumonia, urinary tract infections, or septicemia.
  • 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, uncal herniation, 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 patient with head elevated, avoiding hypothermia and hypercapnia, controlling agitation, and instituting moderate hyperventilation.
  • GI bleeding
  • Electrolytes imbalance
  • Renal dysfunction with renal failure: This occurs in as many as 50% of patients. Therefore, monitoring fluids and renal function tests is important.
    • Kidneys are involved secondary to hepatorenal syndrome (HRS), acute tubular necrosis, drug-induced nephrotoxicity, or prerenal azotemia.
    • 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 in the serum, low plasma osmolarity, high urine osmolarity, and poor nutritional status. Avoid large-volume paracentesis without plasma volume replacement.
    • 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, although one has been shown to reverse HRS.
  • Acid-base disturbances
  • Pulmonary and cardiovascular problems
  • Coagulopathy caused by decreased synthesis of clotting factors by the liver, thrombocytopenia, and abnormal platelet function

Prognosis

  • Acute liver failure in children is a rare but potentially devastating disease.2 Prognostic criteria include patient's age, etiology of liver disease, degree and onset of encephalopathy, serum bilirubin level, prothrombin time (PT) or international normalized ratio (INR), serum creatinine level, factor V level, and arterial pH level.
  • Presence of jaundice for at least 1 week before the onset of encephalopathy is associated with a poor prognosis. Patients with illness lasting longer than 8 weeks before the onset of encephalopathy have a higher likelihood of developing portal hypertensive manifestations such as renal failure. Increased incidence of cerebral edema is associated with illness lasting fewer than 4 weeks before disease.
  • The maximum INR reached during the course of illness is a sensitive predictor of outcome. With an INR of 4 or more, the mortality rate reaches 86%; with an INR of less than 4, it is as low as 27%.
  • Children with fulminant hepatic failure who have severe coagulopathy, prolonged duration of illness prior to the onset of encephalopathy, and lower alanine aminotransferase on admission are more likely to require liver transplantation.3 Early referral to a specialized center for consideration of liver transplantation is vital in these patients.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Atypical presentation of fulminant hepatic failure (FHF) in neonates and young infants accounts for the occasional delay in diagnosis or even in missed diagnosis.

Special Concerns

  • FHF remains a serious disease with a high mortality rate, especially among infants and young children.
  • Special concerns arise because of the following:
    • The exact etiology of FHF remains unknown in a high percentage of patients, especially among infants and young children.
    • An understanding of the exact mechanism of FHF in children that results in massive hepatic necrosis is still needed to provide measures to prevent the progress of the disease.
    • Shortage of organ donors affects survival rate.
 


More on Fulminant Hepatic Failure

Overview: Fulminant Hepatic Failure
Differential Diagnoses & Workup: Fulminant Hepatic Failure
Treatment & Medication: Fulminant Hepatic Failure
Follow-up: Fulminant Hepatic Failure
References
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References

  1. Baker A, Alonso ME, Aw MM, et al. Hepatic failure and liver transplant: Working Group report of the second World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. Jun 2004;39 Suppl 2:S632-9. [Medline].

  2. Cochran JB, Losek JD. Acute liver failure in children. Pediatr Emerg Care. Feb 2007;23(2):129-35. [Medline].

  3. Lee WS, McKiernan P, Kelly DA. Etiology, outcome and prognostic indicators of childhood fulminant hepatic failure in the United kingdom. J Pediatr Gastroenterol Nutr. May 2005;40(5):575-81. [Medline].

  4. Baccarani U, Adani GL, Sainz M, et al. Human hepatocyte transplantation for acute liver failure: state of the art and analysis of cell sources. Transplant Proc. Jul-Aug 2005;37(6):2702-4. [Medline].

  5. Caraceni P, Van Thiel DH. Acute liver failure. Lancet. Jan 21 1995;345(8943):163-9. [Medline].

  6. Dhawan A, Cheeseman P, Mieli-Vergani G. Approaches to acute liver failure in children. Pediatr Transplant. Dec 2004;8(6):584-8. [Medline].

  7. Goss JA, Shackleton CR, Maggard M, et al. Liver transplantation for fulminant hepatic failure in the pediatric patient. Arch Surg. Aug 1998;133(8):839-46. [Medline].

  8. Hattori H, Higuchi Y, Tsuji M, et al. Living-related liver transplantation and neurological outcome in children with fulminant hepatic failure. Transplantation. Mar 15 1998;65(5):686-92. [Medline].

  9. Lee WM. Acute liver failure. N Engl J Med. Dec 16 1993;329(25):1862-72. [Medline].

  10. Suchy FJ. Fulminant hepatic failure in children. Saudi J Gastro. 1996;2(1):34-43.

  11. Treem WR. Fulminant hepatic failure in children. J Pediatr Gastroenterol Nutr. 2002;35 Suppl 1:S33-8. [Medline].

  12. Whittington PF. Fulminant hepatic failure in children. In: FJ Suchy, ed. Liver Disease in Children. Philadelphia, PA: Mosby; 1994:180-213.

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Further Reading

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Keywords

fulminant hepatic failure, FHF, hepatic failure, acute hepatic failure, liver failure, sudden-onset liver failure, hepatic encephalopathy, sepsis, disseminated intravascular coagulation, hyperacute liver failure, liver dysfunction, acetaminophen toxicity, jaundice, subacute liver failure, Wilson disease, autoimmune liver disease, liver transplantation, fetor hepaticus, ascites, cerebral edema, viral hepatitis, Epstein-Barr virus, cytomegalovirus, CMV, paramyxovirus, varicella-zoster virus, parvovirus, adenovirus, hepatitis A virus, HAV, hepatitis C virus, HCV, hepatocellular necrosis, tyrosinemia, hereditary fructose intolerance, galactosemia, neonatal hemochromatosis, congestive heart failure, Hodgkin disease, leukemia

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Contributor Information and Disclosures

Author

Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H, Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, Bushnaq Medical Centre, University of Jordan
Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H is a member of the following medical societies: Royal College of Paediatrics and Child Health, Royal College of Physicians, Royal College of Surgeons in Ireland, Royal College of Surgeons of Edinburgh, and Royal Society of Tropical Medicine and Hygiene
Disclosure: Nothing to disclose.

Coauthor(s)

Dena Nazer, MD, Fellow, Child Protection Center, Children's Hospital of Michigan
Dena Nazer, MD is a member of the following medical societies: Ambulatory Pediatric Association and American Academy of Pediatrics
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, State University of New York, Downstate Medical Center College of Medicine; Distinguished Lecturer, New York Medical College, School of Public Health
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership

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, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

 
 
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