Urinalysis and Body Fluids 6th Edition by Majorie DiLorenzo, Susan Strasinger

Exercise 13

Urine from a newborn with MSUD will have a significant:

A. Pale color

B. Yellow precipitate

C. Milky appearance

D. Sweet odor

Step 1 of 2

MSUD (Maple Syrup Urine Disease):

• MSUD is caused by inborn error of metabolism, inherited as autosomal recessive characteristic

• Failure to inherit the gene, which produces necessary enzyme for oxidative decarboxylation of keto acid will result in accumulation of keto acid in urine, and blood

• Newborns show symptoms of failure to inherit the gene within 1 week

• The disease is characterized by sweet odor, which is similar to maple syrup, because of accumulation of keto acids in urine

Therefore, urine from inborn with MSUD will have “sweet odor”. Hence, the correct answer is option.