About PKU and amino acids

 

 

 

 

(Q AM-06) H.C, a 4 month-old male infant, emigrated with his parents from a Latin American country to the United States a month ago. He was normal at birth but in the past several days a tremor in his extremities has appeared. Last night he presented gross twitching movements in his crib. When you examine the patient, you note a musty odor to the baby’s wet diaper.

 

Immediately you order a screening test for PKU and it results positive.

 

Besides diet restrictions, but warranting an appropriate amount of the essential amino acids, which of the following amino acids you should supplement in higher quantities than in a normal diet, since it is essential for this patient?

 

a)     Alanine

 

b)     Asparagine

 

c)      Aspartate

 

d)     Glutamate

 

e)     Phenyl alanine

 

f)       Serine

 

g)     Tyrosine

 

Answer