A: This is certainly worth mentioning at your baby's next well visit, but if he's developing and growing normally, then you probably don't have anything to worry about. Most of the time these odors subside as your baby's diet expands to include foods other than breast milk and formula, which can cause a baby's pee to take on a distinctive smell when given exclusively. There is a slight chance, however, that this brown-sugar smell could be a sign of maple syrup urine disease (MSUD), a rare, inherited metabolic disorder (it affects only one in every 180,000 babies) that most states screen for at birth. MSUD occurs when a child is born without the enzymes needed to digest certain proteins. Although the most severe form of MSUD can be very serious, most kids with the condition have more mild cases that include symptoms like the sugary pee smell, as well as occasional vomiting, poor appetite, slowed weight gain and sometimes seizures. With treatment (including a protein-restricted diet and sometimes supplements of the vitamin thiamine) and regular checkups, most children live long, fairly healthy lives.
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