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Hepatic Encephalopathy

Hepatic Encephalopathy is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • Reversible altered mental and neuromotor functioning occurring in association with acute or chronic liver disease and/or portal systemic shunting
  • The prominent features are confusion, impaired arousability, and a “flapping tremor” (asterixis).
  • System(s) affected: gastrointestinal (GI); nervous
  • Synonym(s): portosystemic encephalopathy (PSE); hepatic coma; liver coma

Epidemiology

Predominant sex: male = female (reflects prevalence of underlying liver disease)

Prevalence
  • Overt hepatic encephalopathy (HE) occurs in 30–45% of cirrhotic patients.
  • Occurs in all cases of fulminant hepatic failure
  • Present in ~50% of patients requiring liver transplantation
  • Parallels the age predominance of fulminant liver disease: peaks in the 40s; cirrhosis peaks in the late 50s; may occur at any age

Etiology and Pathophysiology

  • There is no defined pathophysiology for the development of HE. Three classifications have been proposed (1):
    • Type A: resulting from acute liver failure
    • Type B: resulting from portosystemic bypass or shunting
    • Type C: resulting from cirrhosis
  • Several metabolic factors implicated in HE based on the failure of the liver to detoxify noxious CNS agents (e.g., ammonia, mercaptan, fatty acids)
  • Increased aromatic and reduced branched chain amino acids in blood may act as false neurotransmitters, possibly interacting with the γ-aminobutyric acid (GABA) receptor and causing clinical symptoms.
  • HE presents most commonly in long-standing cirrhosis with spontaneous shunting of intestinal blood through collateral vessels or surgical portacaval shunts.
  • Asterixis is the inability to maintain a particular posture due to metabolic encephalopathy. Abnormal diencephalic function leads to the characteristic liver flap noted when the arms and wrists are held in extension. Asterixis is also present in patients with uremia, barbiturate toxicity, and some cases of pulmonary disease. As such, asterixis is not pathognomonic for HE.

Genetics
  • Unknown
  • Conditions that predispose an individual to developing chronic liver disease such as cystic fibrosis, α1-antitrypsin deficiency, hemochromatosis, and Wilson disease can contribute to the development of HE.

Risk Factors

In patients with underlying liver disease, precipitating factors include:

  • Infection (overt or occult, including spontaneous bacterial peritonitis [SBP])
  • GI hemorrhage
  • Use of sedative or opiate drugs
  • Fluid or electrolyte disturbance (Na+, K+, Mg2+ most common)
  • Transjugular intrahepatic portosystemic shunt (TIPS—a radiologically inserted shunt to lower portal pressure)—elderly patients and those with worse liver function are at increased risk of HE following TIPS

General Prevention

  • Recognize early signs and seek prompt treatment.
  • Avoid nonessential medications, particularly opiates, benzodiazepines, and sedatives.
  • Consider lactulose therapy as secondary prophylaxis for recurrence of HE (2)[B].

Commonly Associated Conditions

  • May occur as a complication of acute fatty liver of pregnancy
  • Occurs rarely in patients with a portacaval shunt but normal liver function

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Citation

Stephens, Mark B., et al., editors. "Hepatic Encephalopathy." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117471/1.0/Hepatic_Encephalopathy.
Hepatic Encephalopathy. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117471/1.0/Hepatic_Encephalopathy. Accessed July 21, 2019.
Hepatic Encephalopathy. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117471/1.0/Hepatic_Encephalopathy
Hepatic Encephalopathy [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 July 21]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117471/1.0/Hepatic_Encephalopathy.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Hepatic Encephalopathy ID - 117471 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117471/1.0/Hepatic_Encephalopathy PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -