Question
Physiology Question on Regulation Of Kidney Function
A 4-year-old boy of a first-degree consanguineous couple was noted by the parents to have darkening of the urine to an almost black color when it was left standing. He has a normal sibling, and there are no other medical problems. Growth and development to date are normal. Which of the following is most likely to be elevated in this patient?
A
Methylmalonate
B
Homogentisate
C
Phenylpyruvate
D
α-Ketoisovalerate
Answer
Homogentisate
Explanation
Solution
The correct option is (B): Homogentisate.