Hyperammonemia causes and effects US Medical PG Flashcards - Medical Study Cards
Master Hyperammonemia causes and effects with OnCourse flashcards. These spaced repetition flashcards are designed for medical students preparing for NEET PG, USMLE Step 1, USMLE Step 2, MBBS exams, and other medical licensing examinations.
Hyperammonemia causes and effects Flashcard Deck - 10 Cards
Flashcard 1: Hyperammonemia results in excess NH3, which depletes _____, leading to inhibition of the TCA cycle
Answer: -ketoglutarate
Flashcard 2: In the liver, _____ may transfer a molecule of NH3 to the urea cycle, reforming -ketoglutarate
Answer: glutamate
Flashcard 3: What is the most common urea cycle disorder? _____
Answer: Ornithine transcarbamylase (OTC) deficiency
Flashcard 4: Where does the urea cycle occur?
Answer: steps in both the cytoplasm and mitochondria
Flashcard 5: urea cycle
Answer: carbomoyl phosphate synthetase I
Flashcard 6: What is the overall purpose of the urea cycle?
Answer: to get NH4+ from amino acid catabolism into an excretable form
Flashcard 7: carbomoyl phosphate synthetase I
Answer: urea cycle
Extra: N-acetylglutamate
Flashcard 8: In which pathway is ornithine transcarbamylase involved?
Answer: urea cycle
Flashcard 9: In which pathways is carbamoyl phosphate involved?
Answer: de novo pyrimidine synthesis; urea cycle
Flashcard 10: ornithine transcarbamylase (OTC) deficiency
Answer:
Extra: inability to eliminate ammonia somnolence in infant increased orotic acid in blood and urine, hyperammonemia (and symptoms thereof) same as tx for any hepatic encephalopathy i.e. restrict protein in diet, benzoate or phenylbutyrate, lactulose
XR
ornithine transcarbamoylase
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