Which odor is associated with isovaleric acidemia?

Isovaleric acidemia is a metabolic disorder characterized by the accumulation of isovaleric acid in the body due to a deficiency in the enzyme that breaks it down. The condition is known to produce a distinctive odor that is often described as resembling "sweaty feet." This is due to the chemical composition of isovaleric acid, which is similar to certain compounds found in sweat, giving it that particular smell.

In clinical practice, recognizing the specific odor associated with different metabolic disorders can aid in the diagnostic process. Patients with isovaleric acidemia typically have a distinct body odor that may be noted by caregivers or medical professionals, serving as an important diagnostic clue. Understanding these associations helps in the management and identification of metabolic conditions effectively.