Very acidic urine

[vintagesf]

Hello, I was wondering if anyone here experiences very acidic urine (to the point it burns the skin) after overdosing on fructose?
Thanks!

[charlie]

Yes, Megan does, if she has a leak which still frequently happens it burns her if you don't change her quickly, she has sugar problems but not HFI though. But it gets more noticeable the more sugars she has.

[kate]

Yes, my daughter does to the point that it burns her skin and she cries when she goes to the toilet. If I give her lots and lots to drink it seems to dilute the urine enough to make it not hurt when she pees. She hasn't been diagnosed with HFI but is very fructose intolerant.

[tummyache]

I have been reading that higher than normal acidic level in urinalysis is one of the signs of carbohydrate intolerance...HFI

[kate]

I've read that too tummyache. My daughter's genetic tests came back negative for HFI. I don't want to push for tests or further investigations but it is something I've thought about asking the Drs to look into. I wonder whether a urine test before and after eating fructose would show anything? And if it did, whether it would mean anything?

[charlie]

I might be able to give you more info on that Kate once we have been back to London as I think they are going to load Megs up with a sugar and test her blood and urine, not sure which one yet.

[kate]

That would be great Charlie. Megs and Jyoti seem to be remarkably similar. Maybe Megs investigations will help in sorting out Jyoti too.

[nicoleh]

In terms of HFI, acidic urine is unusual. One of the hallmark reactions to fructose in HFI is metabolic acidosis, particularly lactic acidosis (too much lactate and too little bicarbonate in the blood), and one of the 'faults' that the fructose causes in the kidney is inability to adequately acidify the urine to compensate for the acidic blood. So urine pH above 5.5 (too alkaline) when the pH should be below that to get rid of excess acid in the blood. Certainly urinalysis we've had done while H was eating fructose showed VERY acidic 'system' but urine only pH 6, which is way too alkaline in that state.

On the other hand, I also note that DD's nappies are far more horrid to deal with in the evening after sitting in the nappy bucket for the day. They burn your eyes just from the vapours even though our laundry is outside in the fresh air. This is much worse than with my other girls. I don't think it's acid, I think it's ammonia. Ammonia in the urine would certainly burn the skin and smells 'acidic'.

We have just been given a presumptive diagnosis of HFI, pending DNA, but the renal specialist has categorically said she has HFI - the DNA is basically a formality.

On ingestion of fructose, DD's serum bicarbonate drops, anion gap rises, lactate rises. Urinalysis shows protein in the urine, pH above 5.5. Other tests we ordered (but didn't show up as the urine was taken too late after the fructose ingestion) was reducing substance (other than glucose) in the urine, amino acids in the urine and also amino acids (alanine in particular) in the blood but these haven't come back yet so I don't know if they showed up in DD.

if you are accidentally (or deliberately, under medical advice) giving your child fructose, you could do a before and after blood test for these things, and urine taken at practically the same time for comparison. Of course liver transaminases may rise too, though DDs never have.

Urine alone would probably not show much change as it's about urine vs blood, however if you gave a large dose you should find fructose (or any non- glucose reducing substance) in the urine and probably protein and amino acids. A finger prick for blood glucose levels is also helpful in diagnosis. All of these tests need to be done fairly shortly after ingestion (ours was 12 hours and was too late) but I don't know exactly how long. around an hour I think?

Hope that helps,
Nicole

[ukbill]

Fructose hits after about 20 min in my case.. however it might be slowly building up and it takes 20 min to tie up all the phosphate and cause a hypo.

With both oral and injected IV fructose my blood sugar level remained stable (it went up initially with Sucrose) before crashing at the 20 min time marker.

I was really hurting from the IV Fructose from the start, at 20 min I lost consciousness so far as I can remember. They gave me 150g via IV in 15 / 45 seconds. after refusing to reduce the dose.

As I was slipping away they were in a flat panic, shouting and shaking me not to mention slapping my face! I think they might have realised at that point they had the decimal place wrong and should have given me 15g not 150g!

They were trying to ask me what to give me to pull me out.. I was trying to tell them Glucose.. I have no idea if they gave me any or not.

I could not reply properly because my tongue had swelled up like a sausage and their faces were up in the sky at the end of a long dark tunnel.

It was not a good time.

So a blood test after 20 min should show a drop in blood glucose level because all the Phosphate is tied up in the Fructose phosphate stage that Adolaise B normally cleaves to release the phosphate back into the system

[nicoleh]

I just had an appointment with the renal physician and he confirmed that DD was putting a lot of ammonia into the urine, which explains the burn-your-eyes urine vapours from her nappies and may explain the skin burning the OP asked about.

Bill that sounds truly horrible!

[ukbill]

Good example of how not to do the HFI challenge test!.

Took over 6 months before I started to recover properly. Not sure if I have "come back" the same chap.. I know I have not had the same organisational abilities since that test.

The old saying what don't kill you makes you stronger is not true in this case.

The really annoying thing is this was done 24 hours after I had had 150g Sucrose via IV.. and that gave a classic clear HFI result, and had given me a me a severe kicking, so I was already in a very weak state at that point!

I was lucky in that I was in a good physical condition before the test.

I did not try to throw up with the sugar or Fructose .. proving the vomiting reaction to ingested sugars is a Pavlovian reaction / automatic reaction to a poison.

This is why I am so very much against feeding artificial sweeteners / glucose to an HFI child.. it is just wrong headed.

It removes our only natural self defense, and the gains are purely for the parent, the child gains nothing except the mistaken belief that the can eat the same foods as any other "normal" child.

The fact that "normal" children (and adults) should not be eating sweet foods , particularly those containing refined sucrose / fructose anyway, is a side issue.

[nicoleh]

Hi Bill,
the vomiting reaction could also be because it causes instant irritation to the stomach as well.

With DD (who seems rather resistant to vomiting) she will only vomit if she has a significant quantity of fruit or any cane sugar. If it's a smaller quantity or vegetables, she'll only vomit up if I give her breastmilk or other large quantity of liquid with it. like she must be nauseous, but it only comes up if there's a whole lot of liquid to slosh around in there! Otherwise she just gets the hiccoughs. Which is a GREAT first sign that there's something wrong.

the other day I found her sucking on the end of a tube of toothpaste. thank goodness not a sorbitol one - ingredients all look ok but there are plant extracts as flavours and also it's glycerine based. I wasn't sure if she'd get sick from that or not so I spent the next few minutes freaking a little - once she got the hiccoughs though I knew she's probably had enough to bother her but not too badly (if it's bad she bypasses the hiccough and just goes straight to vomit).

my mum (probably HFI) also gets the hiccoughs heaps. anyone else?

[ukbill]

not too often no not me.
I know if I have had something very sweet it rolls about in my stomach like lead!

I remember when I accidentally "downed" a whole glass of full fat coke.. that was not a good evening (after anyway)

By the morning I was OK again but very weak for some time (a few days), and very sore from pulled stomach muscles from all the retching and vomiting. kind of ruined the camping weekend in Llangollen that did.

[clmims]

 As a Senior Citizen with HFI acidic urine can be compensated by calcium or sodium bicarbinate tablets. I have eliminated alchohol and reduced meat intake. READ THE LABELS. I cook a lot from scratch and avoid brightly colored vegitables. I have lost weight my eating less. I am a true genetic HFI with English roots. Reduce the "white stuff" and limit breads to dark "kosher" style rye or dark breads. An Old Guy in San Antonio.

[charlie]

Hi Old guy in San Antonio, welcome to the board, feel free to fill us in on your history a bit more and what you can and cannot eat, we are a varied bunch on here with some still searching for answers so the more diagnosed pictures we can build up the better info this board will give.

Charlie