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

Exercise 37

A newborn develops severe vomiting and symptoms of metabolic acidosis. Urinalysis results are positive for ketones and negative for glucose and other reducing substances.

a. If the urine had an odor of “sweaty feet,” what metabolic disorder would be suspected?

b. If the newborn was producing dark brown urine with a sweet odor, what disorder would be suspected?

c. Would an MS/MS screen be helpful for the diagnosis?

Step 1 of 3

a.

Organic acidemias:

• It includes isovaleric, propionic and methylmalonoic acidemia

• Early severe illness with vomiting along with metabolic acidosis, hypoglycemia, serum ammonia and ketonuria are the symptoms.

Isovaleric acidemia disorder produces urine with sweaty feet odor due to accumulation of isovalerylglycine.