Understanding a Musty Smell in a Newborn's Urine: What Could It Mean?

What could a musty smell in a newborn's urine indicate?

A) Lack of hydration

B) Phenylketonuria (PKU)

C) Urinary tract infection

D) Overhydration

Answer:

The correct answer is B) Phenylketonuria (PKU)

When a nurse changes the diaper on a newborn and notices a musty smell to the infant's urine, this could indicate a metabolic disorder known as phenylketonuria (PKU). PKU is a rare condition caused by a defect in the gene responsible for creating the enzyme needed to break down phenylalanine, an amino acid.

Infants with PKU are unable to properly process phenylalanine, leading to its accumulation in the body. This accumulation can result in the characteristic musty or mousy odor in the urine.

If left untreated, PKU can lead to intellectual disabilities, behavioral issues, and seizures. Early detection and management are crucial in mitigating the effects of PKU and ensuring the well-being of the newborn.