Does anyone elses kids have unexplained odd odors? Jake's urine used to smell like pancake syrup. He does not have MSUD.

Katie's sweat has a sweet odor to it.

I'm starting to really believe Jake had the same metabolic stuff going on as Katie when he was little.

Langan definitely has odd smells, too.

When Langan was on keto, her sweat smelled like someone was smoking marijuana in her skin- I swear. Not that I have much opportunity to smell pot, but it's not a smell you forget. We never figured that one out and it has gone away now that she's been off the diet for so long.

We worried about the mapile syrup urine disease but she was negative also. Her urine smelled really sweet a few weeks ago and I mentioned it to the pediatrician. He said some UTIs cause sweet smelling urine. We did a culture and sure enough she had a UTI. Langan's breath also sometimes smells really nasty- like fish. We are looking into it being a sinus issue. But I still think there's something metabolic going on that they don't have a name for yet.

Sigh.

fish smell sometimes associated with Carnitine.

let'ssee. My son has smelled like:
Fish...carnitine supplements
poop...I Tell you, his hair/skin/sweat/breath when not freshly washed smells like poop. He gets frequent baths.
some odor I can't identify
sickly sweet urine/skin.
also that tart smelling urine that comes from proteinuria.
(his breath has been horrid from various meds in the past.)



and then he also smells like the million other 'additives' he self-applies to his body in the course of a day oh! oops, that's irrelevant to this question....but true.

some of the formulas leave a after effect on the breath, simply becasue of reflux. The maple syrup smell may be something simple like the UTI theory, or dehydration. Lauren's urine smelled syrupy when she wasnt getting enough water and it was concentrated. It looked ok in color, but when examined in the lab along with serum levels, she was slighltyly dehydrated. try some more water to see if that helps.

Alot of sweet smells come from an acidosis...and sometimes can i think indicate ketosis in some kids, cant it? I thought i had heard this at one point in time. A raunchy smell on the breath in particlar can indicate a sinus or throat infection. Sweet smelling sweat, never heard of that one, salty sweat, sometimes indicative of CF. but it gets confirmed with a sweat chloride test...and chances are ya'd know by now.

I asked our geneticst about it. She had never heard of MMA giving off a sweet smell, BUT we sat and brainstormed together, she knows I'm a research hound. Both MSUD (which the kids do NOT have, but Jake did smell like) and MMA (which Katie has a form of and we don't know if Jake had as an infant) they both involve an amino acid called isoleucine, which in whatever kinda process it involves is believed to cause the maple syrup urine smell in MSUD.

So, we came to the theory that possibly Katie smelling sweet is due to the MMA (or more specificly the isoleucine pathway being messed up) and because Jake had a similar odor, though not exactly the same, it is likely he had this form of MMA as an infant.

We know Jake's wasn't UTI's. Though everytime I would ask about the smell they always said, well, it's probably the depakene, it can do weird things to urine. They knew he didn't have MSUD, so they weren't worried.

She said that unexplained (ie- not sinus or carnitor, etc) odd odors are often signs of underlying metabolic disorders.

Katie mostly smells sweet from her neck up and most specifically on one patch of hair that has a different texture and growth pattern than the rest. Jake has this exact same area of hair, but I've never known it to smell sweet, but I may just not have sniffed at him back then you know?

...........Jake's breath always smells like poop! Now I know that one is sinuses!


Wow! Langan smells like pot? How do explain that one to folks !!?!!
No, I've never personally done drugs, but pot is one you just know when you smell it!

Aidan has had breath like COMPOST at times! I think it comes from reflux and also constipation. Luckily, it only happens when he's under the weather. When he was younger he sometimes had ACETONE breath! That concerned me, yet I never could get a doctor to explain or seem to take it very seriously, so I posted here about it.
His bad breath incidents increased after the G-tube.
His urine has a strong smell, at times but not sweet. I think it gets too concentrated sometimes, from borderline dehydration.
Aidan's formula is Vanilla flavored Peptamen Jr, which tastes to me like melted icecream. I'm sure it must give him a sweet smell that I'm too familiar with to detect. I mostly love the way he smells.
Does Jake drink a lot of sweet smelling nutritional supplement drinks?

Jake doesn't have the smell anymore. It was when he was an infant. He was on one of the nasty baby formulas. Can't remember which one. But he didn't tolerate any of them. He projectile vomited. Which come to think of it may be another indication of him having the protein intolerance (MMA) Katie has had breastmilk up until the past couple months and now she's getting maybe 8 oz a day of formula.

Katie's urine has a wicked odd smell to it. Not necessisarily BAD, just weird, but I'm assuming that is from her hyroxocobalimin shots. But her breath is lovely. I love to smell her breath, does that make me weird? Excluding when she drinks the formula.

oh, yeh. I had completely forgotten. Jake's poop smells like moth balls sometimes. Thanks for reminding me, Jake... pe-u! yuck!

Before Paige was ever evein considered as a new infant to have any sort of problems, one thing I always mentioned to the dr.s at her 1 week check up, 6 week check up, and so on was the strong, strong scent of her urine. It wasnt maple syrup sweet, but it wasnt a urine smell either. a year later she was diagnosed with a uti which led to a battery of tests that eventually led us to kidney disease. but thats probably not the case with Jake or Katie. But I mention this because her acid levels are high (can't remember exactly what). It seems like all her tests are always a little off or borderline, when they check her blood or urine. But i didnt realize until this post that I havet noticed that smell w' Paige lately. She is on some meds for her kidneys, Pyridoxine. Maybe that means its working!!