Hyperammonemia: Difference between revisions
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===Types=== | ===Types=== | ||
*Primary | *Primary | ||
**Caused by several [[inborn errors of metabolism]] | **Caused by several [[inborn errors of metabolism]] | ||
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***Normally from acute or chronic [[liver failure]] | ***Normally from acute or chronic [[liver failure]] | ||
====Acquired vs. | ==Clinical Features== | ||
==Differential Diagnosis== | |||
==Evaluation== | |||
===Acquired vs. Congenital=== | |||
*Acquired | *Acquired | ||
**Diseases that result in [[liver failure]] | **Diseases that result in [[liver failure]] | ||
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*Congenital | *Congenital | ||
**Genetic | **Genetic | ||
==Management== | ==Management== | ||
*Treat underlying disorder | |||
==Disposition== | ==Disposition== | ||
*Depends on cause | |||
==See Also== | ==See Also== | ||
Revision as of 10:45, 18 July 2020
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
- Caused by several inborn errors of metabolism
- Characterized by reduced activity of urea cycle enzymes
- Secondary
- Caused by inborn errors of intermediary metabolism (i.e. reduced activity of enzymes that are not part of the urea cycle)
- Normally from acute or chronic liver failure
- Caused by inborn errors of intermediary metabolism (i.e. reduced activity of enzymes that are not part of the urea cycle)
Clinical Features
Differential Diagnosis
Evaluation
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
Management
- Treat underlying disorder
Disposition
- Depends on cause