Hyperammonemia

Revision as of 10:43, 18 July 2020 by Rossdonaldson1 (talk | contribs) (Acquired vs. congenital)

Background

Pathophysiology

  • Acquired: liver failure results in shunting of blood from the liver to the inferior vena cava, decreased filtration of blood and removal of nitrogen-containing toxins by the liver, and then hyperammonemia.
  • Congenital: Defect in one of the enzymes of the urea cycle, which leads to lower production of urea from ammonia.

Types

Primary vs. secondary

  • Primary
  • Secondary
    • Caused by inborn errors of intermediary metabolism (i.e. reduced activity of enzymes that are not part of the urea cycle)

Acquired vs. congenital

  • Acquired
    • Diseases that result in liver failure
    • Medications (e.g. valproic acid overdose)
    • Severe dehydration (from small intestinal bacterial overgrowth)
    • Glycine toxicity (CNS symptoms and nausea)
  • Congenital
    • Genetic

Clinical Features

Differential Diagnosis

Evaluation

Management

Disposition

See Also

External Links

References