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September 17, 2001

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Question from Wilcox, Pennsylvania, USA:

My three year old son, who has had type�1 diabetes for a year and a half and is in very good control, developed a distinct odor (sweeter smelling like almond extract or something close to it) in his urine and body sweat for about a week last month, even though he repeatedly tested negative for ketones and all blood sugars were normal. We have not been successful in getting the doctors to find anything wrong (no testing has been done), and now the smell has returned. Do you have any thoughts or suggestions? We are afraid that something could be wrong, but we are not sure where to start.

Answer:

From: DTeam Staff

The fact that the smell is transient and in an otherwise healthy little boy with type�1A (autoimmune) diabetes suggests a dietary cause including the possibility of trimethylaminuria which is an inability to fully metabolise a normal dietary constituent called choline. The smell is more often described as ‘fishy’ rather than like almonds: the condition is a harmless one, though later on there can be social consequences. Initially the smell is often noted in relation to fish or eggs in the diet, and you might think to ask the doctor about this.

It is easy to diagnose, but it usually takes a special metabolic laboratory to do this. I suspect though that you are going to find some much more simple dietary cause for this.

DOB