Hepatorenal syndrome

Definition
Hepatorenal syndrome is acute kidney failure occurring without other cause in a person with severe liver disease.

Causes, incidence, and risk factors

Hepatorenal syndrome occurs when there is a decrease in kidney function in a person with a liver disorder. The most common symptom is decreased urine production. As a result of reduced elimination of urine, nitrogen-containing waste products accumulate in the blood stream (azotemia).

The exact cause of hepatorenal syndrome is unknown. For some reason, there is drastic reduction in blood flow to the kidneys. The kidney structure remains essentially normal and the kidneys often will instantly function well if the liver disease is corrected (for example, by liver transplantation).

The disorder occurs in up to 10% of patients hospitalized with liver failure. It may be a sign of impending death caused by the accumulated effects of liver damage and kidney failure in people with acute liver failure, cirrhosis or alcoholic hepatitis. It is diagnosed when other causes of kidney failure are ruled out.

Risk factors include cirrhosis, alcoholic hepatitis, acute liver failure, recent abdominal paracentesis, infection, gastrointestinal bleeding, use of diuretics and the presence of orthostatic hypotension (blood pressure which falls when the person rises or suddenly changes position).

Symptoms

     
  • Decreased urine production  
  • Dark-colored urine  
  • Yellow skin  
  • Weight gain  
  • Abdominal swelling  
  • Change in mental status       o Dementia       o Delirium       o Confusion  
  • Coarse muscle movements, jerking  
  • Nausea and vomiting

Signs and tests

A physical examination will not directly reveal kidney failure. However, the exam will usually show signs of chronic liver disease - hepatic encephalopathy, jaundice, ascites, and other signs of liver failure. Deep tendon reflexes are increased and abnormal reflexes may be present, indicating damage to the nervous system. The abdomen is dull when tapped with the tips of the fingers, and often has a visible fluid wave when examined by feel. There may be increased breast tissue, decreased testicular size, lesions (spider telangiectasia) on the skin, or other signs of liver failure.

The following may indicate kidney failure:

     
  • Absent or low urine production, less than 400 cc/day  
  • Very low urine sodium concentration  
  • Increased urine specific gravity and osmolality  
  • Low serum sodium  
  • Fluid retention in the abdomen or extremities  
  • Increased BUN and creatinine levels (in people with liver disease, the BUN and creatinine levels underestimate the severity of kidney dysfunction)

The following may indicate liver failure:

     
  • Low serum albumin  
  • Abnormal PT  
  • Increased ammonia levels  
  • Paracentesis with ascites  
  • An EEG may be performed if signs of hepatic encephalopathy are present

Treatment

Treatment is aimed at improving liver function and to ensuring that circulating blood volume and cardiac output (heart pumping action) are adequate. The disorder is generally treated in the same manner as kidney failure from any cause. All unnecessary drugs (particularly neomycin, NSAIDS and diuretics) are stopped. Dialysis may improve symptoms. There are medications such as octreotide, albumin, and dopamine, which may be used as a temporary measure to improve kidney function.

Surgical placement of a shunt from the abdominal space (peritoneum) to the jugular vein or superior vena cava (called a Levine shunt) may reduce ascites and reverse some of the symptoms of kidney failure. However, surgical shunts are rarely placed, because severe liver disease makes surgery very risky. A non-surgical transjugular intrahepatic portosystemic shunt (TIPS) may be tried in some patients.

Expectations (prognosis)
The probable outcome is poor. Death commonly occurs as a result of secondary infections or hemorrhage.

Complications

     
  • Secondary infections  
  • Bleeding  
  • Hepatic coma  
  • End-stage kidney disease  
  • Fluid overload with subsequent congestive heart failure or pulmonary edema  
  • Damage and failure of multiple organ systems

Calling your health care provider
This disorder most often is diagnosed in the hospital during treatment for a liver disorder.

Johns Hopkins patient information

Last revised: December 5, 2012
by David A. Scott, M.D.

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