A.AS Flashcards
What is the most common cause of hyperammonemia?
Liver disease (e.g., cirrhosis, hepatitis, biliary obstruction).
How does ammonia affect the Krebs cycle?
Ammonia reacts with alpha-ketoglutarate, impairing the Krebs cycle and reducing ATP in neurons.
What are the symptoms of ammonia intoxication?
Tremors, vomiting, slurred speech, blurred vision, cerebral edema, increased cranial pressure, coma.
What are the terminal stage complications of hyperammonemia?
Cerebral edema, increased cranial pressure, death if untreated.
What are the alternative excretion pathways for ammonia?
Sodium benzoate + glycine -> hippurate (urine); phenylacetate + glutamine -> phenylacetylglutamine (urine).
What supplements are used to manage hyperammonemia?
L-arginine and L-citrulline.
What is OTC deficiency?
Ornithine transcarbamylase (OTC) deficiency, a urea cycle disorder causing elevated orotic acid levels.
What is citrullinemia?
A defect in argininosuccinate synthetase, leading to high plasma and CSF citrulline levels.
What is argininosuccinic aciduria?
Absence of argininosuccinase, causing elevated argininosuccinic acid levels.
What is hyperargininemia?
Deficiency of arginase, leading to increased blood and CSF arginine levels.
What are the types of hereditary hyperammonemia?
Hyperammonemia type I (CPSI deficiency), type II (OTC deficiency).
What dietary interventions help manage hyperammonemia?
Protein restriction and ammonia-lowering diets.
What are the treatments for ammonia detoxification?
Sodium benzoate, phenylacetate, phenylbutyrate, protein restriction, dialysis.
What is the role of sodium benzoate in treating hyperammonemia?
Reacts with glycine to form hippurate, excreted in urine to reduce ammonia levels.
What is the role of phenylacetate in treating hyperammonemia?
Reacts with glutamine to form phenylacetylglutamine, excreted in urine.
