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Mystery Illness - Shot in the Dark Here!

Okay folks, maybe I'm desperate and that's okay.  Here's the story.  I brought my sister into the ER here two weeks ago with what I thought was a stomach virus and a declined mental state.  I had seen something similar with my father a month and half earlier when he went into renal failure after having a kidney out due to cancer.  (He unfortunately lost his battle after it metastasized to his brain and skull.  I've seen mental confusion like you wouldn't believe.)  Anyway, my sister quickly declined and a ABG revealed a blood ammonia level of 192.  Liver and kidney function were normal.  CT scan of the brain revealed nothing.  She's had rheumatoid arthritis for the past seven years and a gastic bypass five years ago.  She was swept up the ICU unresponsive and eventually needed to go on a vent, and administered Lactulose, which didn't bring her ammonia down fast enough.  So she underwent a four hour course of dialysis.  This started to bring it down.  In the mean time, her potassium dipped.  Hepatic Encephalopathy ensued (of course).  Her ammonia level came down, potassium came up.  Her blood levels were all normal.  Her MRI of the brain, liver biopsy, EEG, blood tests, and lumbar puncture were all unremarkable.  She was put on an NG tube.  She came out of her unresponsive state a week later.  They took her off the vent three days after that, and she seemed pretty with it, but would be confused from time to time.  They moved her out of ICU and up to a regular room, but her mental state continued to decline.  This was chalked up to ICU Psychosis. Another MRI showed no brain damage, but her mental state was still very bad.  If anyone else has ever been around this, you know how hard it is to see your loved one go through this.   Last night, she started vomiting and spiked a high fever, was sent back to ICU and is back on the vent.  My mother thinks this was due to a kink in the NG tube that had to be straightened.  They are treating her symptoms with antibiotics.  We are still left with no answers as to the cause of the initial incident, if this is a continuation of the incident, a secondary infection or what.  We aren't sure if we should transfer her to another (better hospital), because we don't know what she has or who to see about it.  I know that not every medical incident has an explanation, but was wondering if anyone has ever experienced or even heard of something like this happening.  We're kinda beside ourselves here.  Thanks for reading this incredibly long post.  
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1756321 tn?1547098925
Excerpt from the National Urea Cycle Disorders Foundation (NUCDF): "Risk of Urea Cycle Disorder after Gastric Bypass (Bariatric) Surgery" article...

"There have been several cases of hyperammonemia (elevated ammonia levels) in individuals who have undergone gastric bypass (bariatric surgery). Some of these individuals died shortly after surgery, and some had a steady decline up to four years after surgery that led to coma or death due to elevated ammonia levels.  A number of the individuals were found to have a previously undiagnosed genetic mutation for a urea cycle disorder (ornithine transcarbamylase deficiency) and other cases were considered to have "secondary" or "acquired" urea cycle disorder due to malnourished states.1,2,3"

A comment on the NUCDF website: "My wife went into a coma after gastric bypass surgery. The doctors had no idea what was wrong. One of the ICU doctors took an ammonia level. I called the National Urea Cycle Disorders Foundation and they got an expert in UCD to help my wife's doctors. She was transferred to his hospital and he was able to save her. She still has a lot of problems with her brain from the coma and now has to be treated for the OTC disorder."

UCD means urea cycle disorder and OTC is Ornithine transcarbamylase deficiency.  The NUCDF website goes into treatment options including amino acid formulas (Cyclinex, EAA, UCD I&II), Sodium phenylbutyrate (trade name Buphenyl), L-citrulline (for OTC and CPS deficiency), L-arginine free base (ASA and citrullinemia) and when optimal treatment fails for CPS and OTC deficiency - liver transplantation.

Excerpt from CHEST journal article "Hyperammonemia in the ICU" The causes with [ ] are not listed directly under increased or decreased causes of hyperammonemia. I added more info in { }.  

Table 2. Causes of Hyperammonemia in Adults

Increased Ammonia Production -

Urease producing bacteria (Proteus, Klebsiella)  
Herpes infection

Protein load and increased catabolism:
Severe exercise
Trauma or burns  
Steroid administration  
Gastric bypass
GI hemorrhage
- Increased renal ammonia production  
- Increased splanchnic ammonia production
- Increased peripheral catabolism due to deficiency of essential amino acids

TPN {total parenteral nutrition}

Cancers (multiple myeloma)

Decreased Ammonia Elimination -

Liver failure:
Fulminant hepatic failure

[Trans-hepatic, intrajugular]

Portosystemic shunt (TIPSS)


IEM {Inborn Error of Metabolism}:
Ornithine transcarbamylase deficiency
Carbamyl synthetase deficiency
NAGS {N-acetyl glutamine synthetase} deficiency

[Arginosuccinate lyase deficiency]
[Hyperomithinemia, hyperammonemia, homocitrillinuria]

Lysinuric protein intolerance
Organic acidurias

[Fatty acid oxidation defects]

IHA {Idiopathic hyperammonemia}
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