Pediatric Fulminant Hepatic Failure Clinical Presentation
- Author: Hisham Nazer, MB, BCh, FRCP, , DTM&H; Chief Editor: Carmen Cuffari, MD more...
Fulminant hepatic failure (FHF) affects previously healthy children with no recognized risk factors for liver disease. Children usually present with a hepatitis-like clinical picture and rapid worsening of symptoms. FHF may present in asymptomatic children with Wilson disease.[9, 10, 11]
Jaundice is the presenting symptom in most pediatric FHF patients. A prodrome of flulike illness may precede jaundice. Fever, anorexia, vomiting, abdominal pain, and fetor hepaticus are associated clinical findings. Infants may present initially with poor feeding, irritability, and disturbances in sleep rhythms, with frank features of encephalopathy manifesting only later.
Altered consciousness is also a sign in patients with FHF. Mental changes occur within 2 weeks of the onset of jaundice in most patients. The patient may become somnolent and/or confused and may respond slowly to painful stimuli.
Children with FHF are critically ill, and symptoms and level of consciousness rapidly deteriorate. Over a few days to weeks, the condition progresses to coma, with development of ascites, cerebral edema, and decorticate and decerebrate posturing.
Gastrointestinal bleeding may occur because of severe coagulopathy. The liver size may be normal, small, or large; the liver may shrink with deterioration of the overall general condition of the patient.
Pay special attention to early symptoms and signs of cerebral edema. These include increased muscle tone, arterial hypertension, seizures, agitation, and sluggish pupillary response to light.
FHF is usually defined as the severe impairment of hepatic functions or severe necrosis of hepatocytes in the absence of preexisting liver disease. However, unlike in adults, encephalopathy may be absent, late, or unrecognized in children. Thus, the emphasis in children is placed on the presence of significant coagulopathy in the absence of sepsis or disseminated intravascular coagulation that is not correctable by the administration of parenteral vitamin K within 8 hours.
This leads to the updated definition by the Second World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition, which proposed a more detailed classification and definition of liver failure in children. The group proposed the following definitions for hyperacute, acute, and subacute liver failure in children (all definitions imply the absence of previous liver disease):
Hyperacute liver failure is defined as coagulopathy due to acute liver dysfunction of up to 10 days total duration by clinical criteria (eg, acetaminophen toxicity). Jaundice is frequently clinically absent initially, and encephalopathy varies.
Acute liver failure is defined as coagulopathy due to acute liver dysfunction of more than 10 days but less than 30 days total duration by clinical criteria. Encephalopathy is absent or impossible to recognize, especially in younger patients. If encephalopathy is present, it tends to be preterminal.
Subacute liver failure is defined as coagulopathy due to acute liver dysfunction of more than 31 days but less than 6 months total duration by clinical criteria. Jaundice is almost always present, and encephalopathy often marks preterminal deterioration. It is seen in Wilson disease, autoimmune liver disease, and postmedications. [9, 11]
Hackl C, Schlitt HJ, Melter M, Knoppke B, Loss M. Current developments in pediatric liver transplantation. World J Hepatol. 2015 Jun 18. 7 (11):1509-20. [Medline].
Kirnap M, Akdur A, Ozcay F, Soy E, Yildirim S, Moray G, et al. Liver Transplant for Fulminant Hepatic Failure: A Single-Center Experience. Exp Clin Transplant. 2015 May 30. [Medline].
Gotthardt D, Riediger C, Weiss KH, Encke J, Schemmer P, Schmidt J, et al. Fulminant hepatic failure: etiology and indications for liver transplantation. Nephrol Dial Transplant. 2007 Sep. 22 Suppl 8:viii5-viii8. [Medline].
Jain J, Jain M. Atypical manifestations of viral hepatitis A and E. Trop Doct. 2013 Jan. 43(1):17-8. [Medline].
Kumar KJ, Kumar HC, Manjunath VG, Anitha C, Mamatha S. Hepatitis A in children- clinical course, complications and laboratory profile. Indian J Pediatr. 2014 Jan. 81(1):15-9. [Medline].
El-Ashry R, Malek HA, Ghayaty EA, El-Gendy AA, Darwish A, Al-Tonbary Y. Treatment for hepatitis C virus-induced portal hypertension in leukemic children. Med Oncol. 2013. 30(2):559. [Medline].
Amin MD, Harpavat S, Leung DH. Drug-induced liver injury in children. Curr Opin Pediatr. 2015 Aug 13. [Medline].
Alonso EM, James LP, Zhang S, Squires RH, Pediatric Acute Liver Failure Study Group. Acetaminophen Adducts Detected in Serum of Pediatric Patients With Acute Liver Failure. J Pediatr Gastroenterol Nutr. 2015 Jul. 61 (1):102-7. [Medline].
Nazer H, Ede RJ, Mowat AP, Williams R. Wilson's disease in childhood. Variability of clinical presentation. Clin Pediatr (Phila). 1983 Nov. 22(11):755-7. [Medline].
Nazer H, Ede RJ, Mowat AP, Williams R. Wilson's disease: clinical presentation and use of prognostic index. Gut. 1986 Nov. 27(11):1377-81. [Medline].
Cochran JB, Losek JD. Acute liver failure in children. Pediatr Emerg Care. 2007 Feb. 23(2):129-35. [Medline].
Lee WS, McKiernan P, Kelly DA. Etiology, outcome and prognostic indicators of childhood fulminant hepatic failure in the United kingdom. J Pediatr Gastroenterol Nutr. 2005 May. 40(5):575-81. [Medline].
El-Karaksy HM, El-Shabrawi MM, Mohsen NA, El-Koofy NM, El-Akel WA, Fahmy ME. Study of predictive value of pediatric risk of mortality (PRISM) score in children with end stage liver disease and fulminant hepatic failure. Indian J Pediatr. 2011 Mar. 78(3):301-6. [Medline].
Sanchez MC, D'Agostino DE. Pediatric end-stage liver disease score in acute liver failure to assess poor prognosis. J Pediatr Gastroenterol Nutr. 2012 Feb. 54(2):193-6. [Medline].
Wlodzimirow KA, Eslami S, Abu-Hanna A, Nieuwoudt M, Chamuleau RA. A systematic review on prognostic indicators of acute on chronic liver failure and their predictive value for mortality. Liver Int. 2013 Jan. 33(1):40-52. [Medline].
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. 2004 Jun. 39 Suppl 2:S632-9. [Medline].
Dhawan A, Cheeseman P, Mieli-Vergani G. Approaches to acute liver failure in children. Pediatr Transplant. 2004 Dec. 8(6):584-8. [Medline].
Latif N, Mehmood K. Risk factors for fulminant hepatic failure and their relation with outcome in children. J Pak Med Assoc. 2010 Mar. 60(3):175-8. [Medline].
Alpert O, Sharma V, Cama S, Spencer S, Huang H. Liver transplant and quality of life in the pediatric population: a review update (2013-2014). Curr Opin Organ Transplant. 2015 Apr. 20 (2):216-21. [Medline].
Goss JA, Shackleton CR, Maggard M, et al. Liver transplantation for fulminant hepatic failure in the pediatric patient. Arch Surg. 1998 Aug. 133(8):839-46. [Medline].
Nakazawa A, Nakano N, Fukuda A, Sakamoto S, Imadome K, Kudo T, et al. Use of serial assessment of disease severity and liver biopsy for indication for liver transplantation in pediatric Epstein-Barr virus-induced fulminant hepatic failure. Liver Transpl. 2015 Mar. 21 (3):362-8. [Medline].
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. 2005 Jul-Aug. 37(6):2702-4. [Medline].
Lee HS, Choi GH, Joo DJ, Kim MS, Kim SI, Han KH, et al. Prognostic value of model for end-stage liver disease scores in patients with fulminant hepatic failure. Transplant Proc. 2013 Oct. 45(8):2992-4. [Medline].
Faraj W, Dar F, Bartlett A, Melendez HV, Marangoni G, Mukherji D, et al. Auxiliary liver transplantation for acute liver failure in children. Ann Surg. 2010 Feb. 251(2):351-6. [Medline].
Hattori H, Higuchi Y, Tsuji M, et al. Living-related liver transplantation and neurological outcome in children with fulminant hepatic failure. Transplantation. 1998 Mar 15. 65(5):686-92. [Medline].
Squires RH, Dhawan A, Alonso E, Narkewicz MR, Shneider BL, Rodriguez-Baez N, et al. Intravenous N-acetylcysteine in pediatric patients with non-acetaminophen acute liver failure: A placebo-controlled clinical trial. Hepatology. 2012 Aug 10. [Medline].