OnlineMedEd Shared "Amino Acids - Urea Cycle & Its Disorders" - 4 Picmonics
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Amino Acids - Urea Cycle & Its Disorders
Urea Cycle
- Mitochondria
- N-Acetylglutamate
- Carbamoyl Phosphate Synthetase I
- NH3 + CO2
- Carbamoyl Phosphate
- Ornithine Transcarbamylase
- Citrulline
- Cytosol
- Aspartate
- Argininosuccinate
- Fumarate
- Arginine
- H2O to Urea
- Ornithine
3 mins
Hyperammonemia
- Pathophysiology
- Hereditary Urea Cycle Defects
- Acquired Through Liver Disease
- Inhibits the Citric Acid Cycle
- Signs & Symptoms
- Somnolence
- Slurring of Speech
- Tremor
- Cerebral Edema
- Vomiting
- Blurring of Vision
- Treatment
- Limit Protein in Diet
- Lactulose
- Benzoate Binds Amino Acid for Excretion
- Phenylbutyrate
4 mins
Ornithine Transcarbamylase Deficiency
- Pathophysiology
- Most Common Urea Cycle Disorder
- X-Linked Recessive
- Signs and Symptoms
- Hyperammonemia
- Decreased BUN
- Carbamoyl Phosphate is Converted to Orotic Acid
- Pyrimidine Synthesis Pathway
- Evident in Babies
3 mins
Orotic Aciduria
- Pathophysiology
- Autosomal Recessive
- Defect in UMP Synthase
- De Novo Pyrimidine Synthesis Pathway
- Symptoms
- Increased Orotic Acid in Urine
- Megaloblastic Anemia
- No Response to B12 or Folate
- Non-Hyperammonemic
- Failure to Thrive
- Treatment
- Oral Uridine Monophosphate
2 mins
