February 22, 2003
Question from Mesa, Arizona, USA:
My almost five year old daughter has had type 1 diabetes for over a year and a half, and very so often she will smell sweet. Sometimes we can attribute this to high blood sugars and other times not. Her sweat will smell sweet and so will her urine. I've been researching MSUD, and it seems like a possibility, but I want to know how common is it in children with diabetes. Can it show itself in a child who is older? What are the symptoms?
Maple syrup urine disease (MSUD) is extremely rare and even more rare in association with diabetes. I suspect that what you are smelling are ketones being exhaled from the lungs and would do either a blood ketone or a urine ketone test during those times to see if this correlates. Then the questions is why abnormal ketone levels so often. Your physicians can do a maple syrup urine disease test if there is any other indication.