Urine organic Acids results

[Guest guest]

I had an urine organic acids test done early May and just got the results

back. Can anyone interupt them for me?

It says: showed an elevation of 3-Hydroxyisobutyric Acid, which may be

present with ketosis or 3-Hydroxyisobutyric Aciduria. Recommend correlate

clinically

and repeat with filter paper blood spot for Biotinidase determination if

indicated.

[Guest guest]

I hesitate to say anything about this, as it is best that a doctor address

this, but I wonder if you are on a high fat, low carb diet? The part that

says correlate clinically is standard with an abnormal value. If this had

not been done when I was diagnosed incorrectly many years ago, I would

probably still have an incorrect diagnosis.

I hope you get to see the doctor soon, to put your mind at rest concerning

these results.

laurie

> From: LILQT4U1984@...

> Reply-To:

> Date: Mon, 21 Jun 2004 09:01:58 EDT

> To: , mito

> Subject: Urine organic Acids results

>

> I had an urine organic acids test done early May and just got the results

> back. Can anyone interupt them for me?

>

>

> It says: showed an elevation of 3-Hydroxyisobutyric Acid, which may be

> present with ketosis or 3-Hydroxyisobutyric Aciduria. Recommend correlate

> clinically

> and repeat with filter paper blood spot for Biotinidase determination if

> indicated.

>

>

>

>

>

[Guest guest]

Barbara,

Thank you very much for the good information. Dr. Cohen is out until

July 7th so I'm just trying to see what I can find on my own for now.

[Guest guest]

ALlison, Only your doctors will be able to put your results in

context of your history, but in case you want to read more, I am

posting one link to a Word document about this disorder and one

abstract from PubMed. Interesting that the abstract summary mentions

low carnitine.

Take care,

Barbara

PS The link is long enough that it has wrapped, so probably won't

work without cutting and pasting both segments.

http://www.climb.org.uk/Disorders/Summaries/Hydroxyisobutyric%

20Aciduria%20(3).doc

Pediatr Res. 1991 Oct;30(4):322-6.

3-Hydroxyisobutyric aciduria: an inborn error of valine metabolism.

Ko FJ, Nyhan WL, Wolff J, Barshop B, Sweetman L.

Department of Pediatrics, University of California San Diego, La

Jolla 92093.

3-Hydroxyisobutyric aciduria, a disorder of valine metabolism, has

been found in a boy in whom the clinical picture was that of a

typical organic acidemia with repeated episodes of ketoacidosis

requiring admission to hospital and parenteral fluid therapy, along

with impressive failure to thrive and chronic lactic acidemia. The

excretion of 3-hydroxyisobutyric acid ranged from 170 to 390

mmol/mol of creatinine. The administration of valine increased this

to 18,700 mmol/mol of creatinine and reproduced the clinical picture

of ketoacidosis. Concentrations of free carnitine were low, and

esterified carnitine was elevated. Treatment with carnitine and a

diet restricted in protein appeared to be beneficial.

> I had an urine organic acids test done early May and just got the

results

> back. Can anyone interupt them for me?

>

>

> It says: showed an elevation of 3-Hydroxyisobutyric Acid, which

may be

> present with ketosis or 3-Hydroxyisobutyric Aciduria. Recommend

correlate clinically

> and repeat with filter paper blood spot for Biotinidase

determination if

> indicated.

>

>

>

>

>