Study your flashcards anywhere!

Download the official Cram app for free >

  • Shuffle
    Toggle On
    Toggle Off
  • Alphabetize
    Toggle On
    Toggle Off
  • Front First
    Toggle On
    Toggle Off
  • Both Sides
    Toggle On
    Toggle Off
  • Read
    Toggle On
    Toggle Off

How to study your flashcards.

Right/Left arrow keys: Navigate between flashcards.right arrow keyleft arrow key

Up/Down arrow keys: Flip the card between the front and back.down keyup key

H key: Show hint (3rd side).h key

A key: Read text to speech.a key


Play button


Play button




Click to flip

5 Cards in this Set

  • Front
  • Back
  • 3rd side (hint)
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
Ammonia levels rise above normal (5-50μmol/L)
Neurotoxic effect on the CNS
Ammonia Intoxication Symptoms:
slurred speech
cerebral edema
loss of vision
coma and death
2 Types of Hyperammonemia:
Acquired Hyperammonemia
Hereditary Hyperammonemia
Ornithine Transcarbamoylase Deficiency (OTCD)
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