What is Hyperammonemia
Hyperammonemia is a metabolic disturbance characterised by an excess of ammonia in the blood. It is a dangerous condition that may lead to encephalopathy and death. It may be prima...
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Well, They, (the almighty Drs), have decided that ...
Max629 Nov 02, 2007
Journal Entry for November 2, 2007
Friday, November 2, 2007
Well, They, (the almighty Drs), have decided that it might be possible that the tumor might be playing a bigger part in this confusion mess. My GI Dr has thrown up his hands. It is amazing, 10 months ago He promised we would get through this together. He seemed so sincere and exited about treatment. Now I think he was exited about the rare diagnosis. When things didn... -
I think things are getting worse. Even thoug...
Max629 Sep 17, 2007
Journal Entry for September 17, 2007
Monday, September 17, 2007
I think things are getting worse. Even though my ammonia levels are in the 90s (lot lower then 200s). My confusion is still off and on. The confusion onset is starting to become predictable though. Well sort of. It typically happens when i have to make a decision and especially in a store. Any store. If i make a list, read the list and grab the items without looking at any... -
I'm ok. Just having a hard time ad...
Max629 Sep 04, 2007
Journal Entry for September 4, 2007
Tuesday, September 4, 2007
I'm ok. Just having a hard time adjusting to medicine. I get so frusterated. The medicine makes me so ill. Nothing like chemo, so i feel guilty even thinking about complaining. But having to work under these conditions is very difficult. When my brain is functioning properly i spend so much time in the bathroom, i feel like what's the use!!!!!!!!!... -
Well been on the new stuff for almost ...
Max629 Aug 14, 2007
Journal Entry for August 14, 2007
Tuesday, August 14, 2007
Well been on the new stuff for almost a week now. I havn't been able to take the full dose yet. I keep thowing it up. So i take half in am and half in pm. I've bought some capsules to put this gross stuff in. Hope it works!!!!!!!!! I'll find out tonight.
Karen -
DR has decided to add sodium benzoate to my list o...
Max629 Aug 02, 2007
Journal Entry for August 2, 2007
Thursday, August 2, 2007
DR has decided to add sodium benzoate to my list of meds. This was at my request. The few articles i could find on the web all indicated it to be effective along with the enulose and 220mg of Zinc every day. Since this is such a mega dose of a specialty item there is only one pharmacy in town that will consider filling it. So i will now be going to 3 different pharma... -
You know, what's really frustrating? I...
Max629 Jul 31, 2007
Journal Entry for July 31, 2007
Tuesday, July 31, 2007
You know, what's really frustrating? I'll be going about my day, feeling great, no problems with thougt process, then all of a sudden BAM my thoughts are so jumbled that i freeze. It's like my mind get's over loaded and flashes into a blank screen. This happens in the middle of conversations, when I'm troubleshooting networks, playing with my kids, watching tv, it ... -
Today is one of my better days. I made it ou...
Max629 Jul 30, 2007
Journal Entry for July 30, 2007
Monday, July 30, 2007
Today is one of my better days. I made it out of the house with my daughter and everything i need for the day. I also remembered to drop her off at the right place. Still having "word finding difficulty" but not as bad. If I keep my protein intake at <10g per meal and take enulose 4x per day I am less likely to get confused. This is a good thing, but the...
Journal Entry for November 2, 2007
Friday, November 2, 2007
Well, They, (the almighty Drs), have decided that it might be possible that the tumor might be playing a bigger part in this confusion mess. My...
Hyperammonemia Information
Hyperammonemia is a metabolic disturbance characterised by an excess of ammonia in the blood. It is a dangerous condition that may lead to encephalopathy and death. It may be primary or secondary.
Ammonia is a substance that contains nitrogen. It is a product of the catabolism of protein. It is converted to the non-toxic substance urea prior to excretion in urine by the kidneys. The metabolic pathways that synthesise urea are located in mitochondria. The process is known as the urea cycle, which comprises several enzymes acting in sequence.
Primary hyperammonemia is caused by several inborn errors of metabolism that are characterised by reduced activity of any of the enzymes in the urea cycle. Secondary hyperammonemia is caused by inborn errors of intermediary metabolism characterised by reduced activity in enzymes that are not part of the urea cycle (e.g .Propionic acidemia, Methylmalonic acidemia) or dysfunction of cells that make major contributions to metabolism (eg hepatic failure).
In all cases Hyperammonemia results from defects of the Urea cycle. The following list includes such examples:
- hyperammonemia due to ornithine transcarbamylase deficiency
- hyperinsulinism-hyperammonemia syndrome
- hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
- hyperammonemia due to N-acetylglutamate synthetase deficiency
- hyperammonemia due to carbamoyl phosphate synthetase i deficiency
- hyperlysinuria with hyperammonemia
See more Hyperammonemia Information.
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