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
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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
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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
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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
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2 mins

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