Maple Syrup Breath??

Our 8 y/o daughter has very occasionally had breath that smells like maple syrup. It's happened about 3 or 4 times over the past year, as clearly as we can figure. Her urine smells normal - no sweet smell; her perspiration is not abnormal either. The last time, we caught a slight whiff of the smell, and had her drink some water, which appeared to clear it. The time prior to that (several days before), she had been crying and upset over the fact we were making her go to her choir class, and she carried on for about 1.5 hours. (She's a very intense kid!) It was morning, she had not eaten/drank anything, and had very little to eat or drink the night before.

Her urine was checked at her pediatrician yesterday - no UTI, sugar levels fine. Our pediatrician says it doesn't sound like MSUD. Our dd is a healthy 8-y/o, no history of seizures or other issues, with above-average intelligence (she was recently accelerated a grade in a gifted school), and normal physical development. She was newborn-screened for MSUD.

As her mother, though, I'm worried. Does this occasional maple syrup smell on her breath seem to indicate anything we should look for? Can it be caused by dehydration/tantruming? Can it be, well, nothing, really?

Thank you. I'm becoming a nervous wreck over this one!

[Deanna L Aftab Guy, MD]

You are asking about maple syrup urine disease which is a metabolic disorder in which the children are not breaking down certain amino acids, in this case the branched chain amino acids like leucine, isoleucine and valine, they are detected in the urine,
As far as the breath you got me!!! Ultimately the newborn screen does not usually test for this rather homocysteinuria, so if you are really concerned blood and urine for organic acids is the ultimate test.
If she is normally developing I would monitor her with your physician. Other foods that smell like this include fenugreek-the herb used to make fake maple syrup also ironically enough stimulate breast milk-what a combo of function!
Hope this help, if you are worried start with urine test and consider blood if urine screen negative, the down side is that it is expensive and a send out but may calm your nerves.

[KatieClipsey]

I'm puzzled - why did you mention homocysteinuria? are you suggesting this smell on her breath indidcates homocysteinuria could be an issue?

Thanks!

[KatieClipsey]

After re-reading my original post, I may not have been clear. our daughter is 8 years old, and the ONLY "symptom" is this very occasional smell on her breath. Our pediatrician checked her urine sugar levels - they're normal. He feels this "syptom (symptom)" is interesting to note, but of no real importance - that it's most likely brought about due to dehydration - especially after "tantrumming" for an hour!
    We just want to make sure we're not overlooking somehting we haven't thought of.
     Thanks again.

[Deanna L Aftab Guy, MD]

I was just summarizing the concerns that may come up from your original question, I agree with your pediatrician especially if the urine and blood are normal and she is growing fine. Maple syrup urine disease is not usually tested for at birth, so if there is a doubt in your doctor's mind they do need to test, but this sounds very unlikely