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5 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Cystinuria
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Inherited Disorder
Results in cystine, ornithine, arginine, and lysine in the urine 1 in 7000 individuals causes precipitation of cystine to form kidney stones Tx: Oral hydration |
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Glucose Alanine Cycle
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Primarily used by muscle as a transport for ammonia
(There are two: other is glutamine synthase transport system) Transamination of pyruvate to alanine Alanine transported to the liver and converted back to pyruvate Pyruvate undergoes gluconeogenesis to produce glucose to be returned to the plasma |
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Glutamine Synthetase Transport System
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Transport for ammonia
(There are two: other is glucose alanine cycle) Glutamine synthetase combines glutamate with ammonia to form glutamine Glutamine through the plasma arrives at the liver and is deaminated |
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Hyperammonemia
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Ammonia levels rise above normal (5-50μmol/L)
Neurotoxic effect on the CNS Ammonia Intoxication Symptoms: tremors slurred speech somnolescence vomiting cerebral edema loss of vision coma and death 2 Types of Hyperammonemia: Acquired Hyperammonemia Hereditary Hyperammonemia |
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Ornithine Transcarbamoylase Deficiency (OTCD)
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X-Linked
Decreased ability to use urea as a nitrogen disposal mechanism hyperammonemic encephalopathy Toxic levels of ammonia in the brain Brain swelling due to glutamine accumulated in astrocytes Lethargy, Vomiting Siezures All with neonatal comma have brain damage Intensive therapy: IQ closer to normal Low Protein Diet Drugs to help clear ammonia Liver Transplant Gene Therapy |
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