Tuesday, June 07, 2005

Follow-up to Coma in an ICU patient

Excellent comments and ideas on the surgery unmasking or leading to a metabolic disorder.
The patient had developed a similar episode after surgery. Prior to surgery she had the standard high-fat, high-starch obesity-related diet. After the surgery she was asked to follow a relatively higher-protein, low-cal and certainly low starch, diet. This unmasked an Ornithine Transcarbamylase deficiency (she had already been tested by the time of this presentation). This leads to high ammonia (hers was real and remained >200-300 prior to transfer to us) levels that worsen in catabolic states (her pneumonia) and with high protein intake (like her post surgery diet).
Treatment includes a steady intake of calories/carbs to prevent further muscle breakdown, lactulose is only partially effective since this is truly endogenous and not from translocation, and aceptors of nitrogen / urea compounds.
We did manage to bring down the ammonia with these measures and she actually had an improvement in MS with eye opening to verbal stimuli and more vigorous withdrawal to stimuli.
Unfortunately, with her very poor performance status and malnutrition, she became septic, eventually developed renal failure, ALI and MOSF and expired.

3 comments - CLICK HERE to read & add your own!:

ACR, MD said...

LOL. Ornithine Transcarbamylase deficiency. Ok, I don't feel so bad I didn't get that one. :)

Jennings said...

That was my second guess actually...

Baleeiro said...

UpToDate actually has a nice card on protein and urea metabolism enzyme deficiencies including OTC and it also has specific therapies. The clue here was the high ammonia with poor nutrition and no primary liver disease :)