why does my baby smell like maple syrup

Hey Shari,
It may be nothing more than that your daughter needs more vegetables in her diet, because the smell can be from eating too much protein, but there is another possible cause that you should have your pediatrician check out. See below: "Maple syrup urine disease or MSUD refers to a rare inherited metabolic condition. The first form results from a deficient enzyme (branched-chain alpha-keto acid dehydrogenase, BCKD) necessary for the breakdown of the amino acids leucine, isoleucine, and valine. Without this enzyme, these amino acids build up to toxic levels in the body. If left untreated, this leads to brain damage and progressive nervous system degeneration in infants. Babies that suffer from the mild form have a sugary smell. Adults may have a burned sugar smell to urine. There second form responds well to the vitamin thiamine.


It is reported that the patients smell like caramel, maple syrup or have a malty odor. " My son has a metabolic disorder. Most of these are treatable. The one that causes a maple syrup smell is very treatable. It is good you're seeing genetics--are you seeing them very soon? If you can find out what abnormalities your pediatrician found in the blood and the urine I might be able to help you more. Ask for a copy of the labs. They have to give them to you. I learned (eventually) that it is always good to have a copy of the labs myself. I assume they found something on his urine organic acids that indicated a metabolic condition. Does your son seem healthy? Is he meeting his milestones? Growing well? Eating well? I think that he is 14 months before you see this is a very good sign. It would indicate, I believe without seeing the labs and realizing this is different than what my son has, that your son must have some metabolic ability in the area that is affected that has protected him.


Or that he has a milder condition. I'm not sure what they might be thinking since it is not his urine that smelled. But if you can get the labs I might be able to tell you more. By my first thoughts were MSUD (a varient form with onset later) or diabetes. MSUD would usually impact urine but it could come through sweat too. If you smell a diaper after the urine has dried do you smell the maple syrup smell? Diabetes would add a sweet smell to the breath and even the skin (does his skin taste sweet? ). I'm assuming more in the metabolics area because your doctor should have been able to handle diabetes or would have at least started treatment I would think and I suspect they did a urine organic acids screen and found something.


It is possible that a child in metabolic decompensation of another sort could smell sweet as they would have acidosis but your son would have been sick and your ped. would have hospitalized him. I hope you're not waiting for genetics; your son needs to be seen immediately. The danger with this stuff is not treating it. In the meantime don't let him fast (go overnight without eating or too long during the day) and protect him from illness. Both those can trigger decompensation if he should have a metabolic disorder. Has he been sick recently? Started sleeping through the night? Has his diet changed recently--did he for example start eating more solids? Did the doctor tell you to watch for anything or to change anything?

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