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5 Cards in this Set

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Cystinuria
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
Glucose Alanine Cycle
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
Glutamine Synthetase Transport System
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
Hyperammonemia
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
Ornithine Transcarbamoylase Deficiency (OTCD)
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