urea cycle metabolism Flashcards

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1

urea cycle occurs in which cells

  1. hepatocytes
  2. kidney
  3. small intestine

2

enzyme defects in he urea cycle

  1. CPS
  2. OTC
  3. AS
  4. AL
  5. AGR
  6. in cps there was argent news about al octopus that it killed an aspiring student in the monster academy

3

enzyme defects outside the urea cycle

  1. HHH
  2. LPI
  3. NAGS
  4. hahaha that little pretty important note is about the nasez gas used in wars

4

monitoring

  1. symptoms
  2. growth
  3. ammonia
  4. essential AA
  5. Hb
  6. HTC
  7. albumin
  8. prealbumin
  9. total protein

5

treatment in acute in neonatal

  1. NPO
  2. no nitrogen intake for 24-48 hrsa
  3. IV fluids
  4. arginine hydrochloride 0.2-0.8 g/kg
  5. sodium benzoate 0.25 g/kg
  6. sodium phenylacetate 0.25 g/kg
  7. glucose 10% + intra lopids 5-30ml/kg > 1-2 hrs
  8. EN & PN BCAA
  9. B6 5mg
  10. folic acid 0.1 mg
  11. mannitol
  12. dialysis

6

chronic intervention

  1. low protein , HBV, 100% digestible, AA mixture
  2. supplements BCAA
  3. breast milk
  4. limited infant formula
  5. special formula > AA, FA, CHO, vit, minerals
  6. arginine, citrulline, fluoride supplements
  7. sodium benzoate, sodium phenylacetate
  8. additional fluids , energy
  9. 1% linolic acid
  10. 2% alpha linolic
  11. iron, copper, zinc, calcium, B, beta carotenes, C