Glycogen storage disease type I is due to a deficiency of the _____ enzyme, which results in _____.
a. glucose-6-phosphatase, hyperglycemia
b. glucose-6-phosphatase, hypoglycemia
c. glucokinase, hypoglycemia
d. glucokinase, hyperglycemia
Q. 2Those with fructose intolerance should avoid which of the following sugars?
a. fructose, sucrose, and sorbitol
b. fructose, glucose, and malitol
c. frucotse, sucrose, and zylitol
d. fructose, raffinose, and glucose
Q. 3Which of the following nutrients are individuals with galactosemia most likely to be deficient in?
a. calcium and vitamin D
b. phosphorus and vitamin A
c. vitamin D and phosphorous
d. magnesium and calcium
Q. 4In galactosemia the defective enzyme, uridyltransferase, is responsible for converting _____.
a. galactose to glucose-6-phosphate
b. galactose-1-phosphate to glucose-1-phosphate
c. glucose-6-phosphate to fructose-6-phosphate
d. galactose to galactitol
Q. 5Which of the following amino acids becomes conditionally essential for those with urea cycle disorders, and thus should be supplemented in their diet?
a. valine
b. arginine
c. alanine
d. histidine
Q. 6Which of the following urea cycle enzyme deficiencies is inherited as an X-linked dominant trait that is usually lethal in males?
a. argininosuccinic acid synthase
b. carbamyl phosphate synthase I
c. ornithine transcarbamylase
d. arginase
Q. 7A block in the urea cycle can result from a deficiency in any of these enzymes except:
a. glucokinase.
b. carbamyl phosphate synthetase I.
c. arginase.
d. argininosuccinic acid lyase.
Q. 8The urea cycle resides primarily in the:
a. small intestine.
b. kidney.
c. red blood cells.
d. hepatocytes.
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