Difference between revisions of "Hyperammonemia"

(Acquired vs. congenital)
 
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===Types===
 
===Types===
====Primary vs. secondary====
 
 
*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. congenital====
+
==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
 
==Clinical Features==
 
 
 
==Differential Diagnosis==
 
 
 
==Evaluation==
 
 
  
 
==Management==
 
==Management==
 
+
*Treat underlying disorder
  
 
==Disposition==
 
==Disposition==
 
+
*Depends on cause
  
 
==See Also==
 
==See Also==

Latest 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
  • Secondary
    • 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

See Also

External Links

References