$Unique_ID{BRK03816} $Pretitle{} $Title{Hepatorenal Syndrome} $Subject{Hepatorenal Syndrome Hepato-renal Syndrome HRS } $Volume{} $Log{} Copyright (C) 1989, 1990 National Organization for Rare Disorders, Inc. 664: Hepatorenal Syndrome ** IMPORTANT ** It is possible that the main title of the article (Hepatorenal Syndrome) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Hepato-renal Syndrome HRS General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Hepatorenal Syndrome is a progressive type of kidney (renal) failure which occurs as a result of severe LIVER disease. It is characterized by acute kidney (renal) failure that is NOT caused by primary KIDNEY dysfunction. Symptoms such as yellowing of the skin (jaundice) and urination problems occur in the two types of this liver disease. Symptoms The blood circulation of Hepatorenal Syndrome patients has unique features. The amount of blood the heart circulates to the rest of the body (cardiac output) is above normal. The arteries that circulate oxygenated blood from the lungs to the rest of the body (systemic circulation) widen. The arteries of the kidney narrow (increase in kidney vascular resistance) causing a decrease in the blood flow through the kidney. There appear to be two types of Hepatorenal Syndrome. Acute kidney failure and yellowing of the skin (jaundice) are common to both. Acute kidney failure is characterized by a rapid onset, decreased urination (oliguria), and possibly proteins or red blood cells in the urine and excess nitrogenous bodies in the blood (azotemia). Jaundice may be accompanied by dark urine, and an enlarged and tender liver. Loss of appetite, fever, tiredness, and weakness may also occur. One type of Hepatorenal Syndrome is characterized by excess watery fluid in the spaces between the tissues and organs in the abdomen causing swelling (ascites), yellowed skin (jaundice), and rapidly advancing renal failure. The brain may be affected (hepatic or portal-systemic encephalopathy) possibly causing slight changes in judgement and other intellectual processes, personality changes, inappropriate behavior, depression, and sleep disturbances. The other type of Hepatorenal Syndrome is characterized by less jaundice, slower progressive kidney failure, and no hepatic encephalopathy. Causes Kidney failure in Hepatorenal Syndrome is caused primarily by liver disease. It is NOT due to a primary abnormality or dysfunction of the kidney. Hepatitis, advanced cirrhosis, obstructive jaundice, liver cancer, and tumors of the bile ducts have also been reported to cause Hepatorenal Syndrome. In many patients, an infection or gastrointestinal bleeding can be identified as a precipitating factor that brought about the Hepatorenal Syndrome. However, a precipitating factor cannot be found in the rest of the cases. Affected Population Hepatorenal Syndrome occurs equally in males and females with severe liver disease. Related Disorders Symptoms of the following disorders can be similar to those of Hepatorenal Syndrome. Comparisons may be useful for a differential diagnosis: There are many causes of acute kidney failure: abnormalities of the blood vessels of or leading to the kidney; abnormalities of the glomeruli of the kidneys due to infections or diseases such as Goodpasture's Syndrome, Polycystic Kidney Disease, or Wegener's Granulomatosis; acute interstitial nephritis (inflammation of the kidney) commonly related to drugs or infections; intratubular obstruction; or acute tubular necrosis. An underlying problem in the kidney distinguishes these disorders from the Hepatorenal Syndrome. (For more information on these disorders, choose "Goodpasture", "Polycystic Kidney", or "Wegener" as your search terms in the Rare Disease Database.) Therapies: Standard It is important to distinguish the Hepatorenal Syndrome from the many other causes of acute kidney failure since the causes and therapies differ. Treatment of Hepatorenal Syndrome is primarily aimed at correcting the unusual features of the blood circulation. Three methods have been used: Head-Out Water Immersion, Paracentesis, and Peritoneovenous (PV) Shunting. Head-Out Water Immersion involves immersing the patient in water, leaving the head out. This redistributes the blood from the arms and legs to the trunk. Paracentesis is a surgical procedure to remove the excess fluid in the abdomen (ascites). Under carefully controlled conditions, this procedure may benefit some patients. Peritoneovenous (PV) Shunting involves surgically placing a tube (a catheter) in the abdominal cavity that drains the ascitic fluid into the blood circulation. Other treatment is symptomatic and supportive. Therapies: Investigational Liver transplants have been used to treat Hepatorenal patients who do not respond to other therapies. Researchers are investigating the effectiveness of introducing fresh frozen plasma into the patient's vein to treat Hepatorenal Syndrome. There is extensive ongoing research in determining the roles of drugs such as atrial natriuretic peptide, calcium channel blockers, prostacyclin, vasodilatory prostaglandins, the liver hormone glomerulopressin, and the surgical procedure lumbar sympathectomy in the treatment of acute kidney failure and the Hepatorenal Syndrome. Current research on liver diseases will hopefully shed more light on the understanding of Hepatorenal Syndrome. This disease entry is based upon medical information available through January 1990. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Hepatorenal Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 American Liver Foundation 1425 Pompton Ave. Cedar Grove, N.J. 07009 (201) 857-2626 (800) 223-0179 Children's Liver Foundation 14245 Ventura Blvd. Sherman Oaks, CA 91423 (818) 906-3021 National Kidney and Urologic Diseases Information Clearinghouse Box NKUDIC Bethesda, MD 20892 (301) 468-6345 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 187-194, 197, 757. THE HEPATORENAL SYNDROME: P.S. Kellerman & S.L. Linas; AKF Nephrology Letter (November, 1988: issue 5(4)). Pp. 47-54. PATHOPHYSIOLOGY OF THE HEPATORENAL SYNDROME AND POTENTIAL FOR THERAPY: M. Levy; Am J Cardiol (December 14, 1987: issue 60(17)). Pp. 66I-72I.