Pediatric Fulminant Hepatic Failure Clinical Presentation

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

History

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. [11, 12, 13]

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.

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Physical Examination

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.

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Classification

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. [20] 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. [11, 13, 14]

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