Hyperammonemic Encephalopathy Syndrome Due to Urinary Bladder Distention and Infection

  • R. R. Varma
  • J. W. Schenck
  • A. Goswami
Part of the Experimental Biology and Medicine book series (EBAM, volume 22)


The role of ammonia in the pathogenesis of hepatic encephalopathy has been widely investigated. Hyperammonemia and cerebral dysfunction without liver disease or portal hypertension, or both can be due to a variety of causes which include acquired or congenital deficiency of Kreb’s urea cycle enzymes such as ornithine transcarbamylase. One of the well established acquired forms is that of Kapilla-Reye’s syndrome (1). Other causes include ureterosigmoidostomy performed to divert urinary flow to the colon and valproic acid toxicity. We have previously reported an interesting syndrome in a patient with hyperammonemic encephalopathy caused by infection without liver disease in a neurogenic bladder. The urinary tract infection (2) was caused by a urease producing organism. Veins from the urinary bladder form plexus on the urinary bladder surface and drain via internal iliac vein into the inferior vena cava, thus bypassing the portal circulation.


Hepatic Encephalopathy Urethral Stricture Neurogenic Bladder Ornithine Transcarbamylase Blood Ammonia Level 
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Copyright information

© The Humana Press Inc. 1989

Authors and Affiliations

  • R. R. Varma
    • 1
  • J. W. Schenck
    • 1
  • A. Goswami
    • 1
  1. 1.Medical College of WisconsinMilwaukeeUSA

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