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The Etiology and Diagnosis of Congenital and Acquired Hyperammonemia

Author: Dana Bartlett, RN, BSN, MSN, MA, CSPI
(Click author's name for bio)

CE: 2 hours Pharmacology: 0.5 hour
Rated 4.8 out of 5.0 based on 137 reviews

Course Summary

Ammonia is a normal metabolic byproduct that can become toxic when the serum ammonia level becomes abnormally high. Hyperammonemia is an acquired or congenital disorder that can lead to neurologic complications ranging in severity from mild and reversible to severe and permanent. Liver cirrhosis is a frequent cause of acquired hyperammonemia, and it can result from medications like valproic acid. Congenital hyperammonemia, like urea cycle disorder, is rare. The cause of hyperammonemia is not always known. Irreversible neurological damage and death can result due to cerebral edema and glutamine synthetase activity, and it affects the activity and functioning of neurotransmitters like ?-aminobutyric acid (GABA), glutamine, and monoamines, and elevated glutamine levels cause cell damage, inflammation, the formation of reactive oxygen species, and other harmful pathologic processes. Whether hyperammonemia is acute or chronic, the age of the patient, and the speed at which the ammonia level increases need to be considered. A cirrhotic liver disrupts urea cycle functioning, and it can cause portosystemic shunting as well. Patients who have cirrhosis have a high risk of infection, and overt hepatic encephalopathy was found to be an independent risk factor for de novo infections.

Course Format


Course Syllabus

  1. Introduction
  1. Acquired and Congenital Hyperammonemia
  1. Ammonia Production
  1. Adverse Effects of Hyperammonemia
  1. Neurological Effects
  2. Sarcopenia
  3. Liver Damage and Immune System Function
  1. Causes of Hyperammonemia
  1. Liver Disease and Hyperammonemia
  1. Acute Liver Failure
  2. Chronic Liver Failure and Cirrhosis
  3. Hepatic Encephalopathy
  1. Hyperammonemia and Overt Hepatic Encephalopathy
  1. Hepatic Encephalopathy
  1. Lactulose
  2. Antibiotics
  3. Other Agents
  1. Valproic Acid and Hyperammonemia
  1. Risk Factors and Clinical Presentation
  1. Other Causes of Hyperammonemia
  1. Measuring Serum Ammonia
  1. Timing and Patient Selection
  1. Serum Ammonia: Obtaining and Handling Blood Specimen
  1. Variables Affecting the Serum Ammonia Level
  1. Summary


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