Case study- Ammonia Intoxication-A 2-year-old girl was seen in the emergency room ..

Question of the day

A 2-year-old girl was seen in the emergency room for vomiting and tremors. The elevated plasma ammonium ion concentration was 200 μM/L (normal 11-40 μM/L). Metabolic screens of blood and serum were ordered and were remarkable for an elevation in the amino acid arginine in serum. Which of the following enzymes would most likely cause an elevation in the plasma Arginine levels?

A. Carbamoyl phosphate synthetase I

B. Carbamoyl phosphate synthetase II

C. Ornithine transcarbamoylase

D. Arginase

E. Argino succinic acid lyase


Figure- Structure of Arginine

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1 Response

  1. Arginase deficiency does’t allow urea to separate out from Arginine.Ornithine fails to separate.It does’t enter mitochondria of liver cells to continue urea cycle.Amino molecules fails to enter urea cycle causing ammonia intoxication.

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