Aight, how good are you all really?

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toughlife

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Another case for the Anesthesia X files

23 y/o female who was scheduled radiofrequency ablation for presumed paroxismal ventricular tachycardia which was noted to occur several times postoperatively. Patient had undergone three surgeries in the past including knee arthroscopy, sinus surgery for chronic sinusitis and neurostimulator for bladder spasticity. After all these surgeries patient had been noted to develop severe weakness and respiratory distress which would require reintubation and ICU admission.

During her scheduled RFA, EP was unable to unmask said ventricular arrythmia and no ablation was performed. During emergence, patient again developed generalized weakness with inability to ambulate, respiratory distress and arrythmia unmasked again. Patient admitted to ICU.

What ya thinking?

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Another case for the Anesthesia X files

23 y/o female who underwent radiofrequency ablation for presumed paroxismal ventricular tachycardia which was noted to occur several times postoperatively. Patient is s/p knee arthroscopy, sinus surgery and neurostimulator for bladder spasticity.
Also postop, pt noted to develop severe weakness and respiratory distress requiring ICU admission.

Not clear why she needed a stimulator to begin with, but...

I would do:

Supportive care
Good history, good exam
Check for MS or Guillan Barre
Neuro consult
MRI/CT of spine to check for hematoma, abscess, granuloma, stimulator migration, or brainstem infract?
 
severe weakness and respiratory distress:

Surgery went without complications otherwise:

Myasthenia Gravis
Atypical Pseudochoinesterase
Inadequate reversal
aspiration
laryngospasm
Multiple Sclerosis

Nab a CXR, grab an ABG and let her chill on the vent till ya figure it out.

She following commands? Anything unusual happen?
 
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goddammit!!

I spend about 2 hours looking up crap and NEVER even thought about familial hypokalemic periodic paralysis.

Nice work Mil.
 
Thats a better history than before.
 
familial hypokalemic periodic paralysis

Nice job. This is like Jeopardy of Anesthesiology. Ever seen this in private practice undiagnosed? What about the rest of her family? Did you actually guess this "cold" or did you have a reference/differential diagnosis to look at?

Blade
 
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Nice job. This is like Jeopardy of Anesthesiology. Ever seen this in private practice undiagnosed? What about the rest of her family? Did you actually guess this "cold" or did you have a reference/differential diagnosis to look at?

Blade

That was actually the first thing that entered my mind.....It sounded like a zebra, so I thought about zebras...and as noted above...we still don't have the actual answer.
 
does everybody do ablations under GA? we don't (not always, at least).
 
Well i guess we know who is really good...
familial hypokalemic periodic paralysis :confused: never heard of that one now i'm going to be waiting on one for the rest of residency :laugh:
 
sorry but it is not familial hypokalamic periodic paralysis. Keep thinking. I will give you more clues. On further interview, parents disclosed patient had had "allergic reactions" to the anesthetic and with everytime the patient had received propofol and sevoflurane but they were not sure which one was the culprit.


I am actually writing this case up.
 
propofol infusion syndrome? mutant mitochondria extremely sensitive to propofol

sodium channel blockade from propofol?

Maybe it wasn't even that. Maybe she had low, or poorly working, acetylcholinesterase and every time she was reversed she became weak.
 
propofol infusion syndrome? mutant mitochondria extremely sensitive to propofol

sodium channel blockade from propofol?

Maybe it wasn't even that. Maybe she had low, or poorly working, acetylcholinesterase and every time she was reversed she became weak.


There ya go! Mutant mitochondria
 
propofol infusion syndrome? mutant mitochondria extremely sensitive to propofol

sodium channel blockade from propofol?

Maybe it wasn't even that. Maybe she had low, or poorly working, acetylcholinesterase and every time she was reversed she became weak.


WOW.

Nice call.
 
cool....even MORE of a zebra....this mutant mitochondria thing is something I know absolutely nothing about....

However, I believe you're calling it by a wrong name...

Propofol Infusion Syndrome has been described in the CCM literature since the late 90's???

Usually peds ICU patients with sepsis require treatment with vasactive drugs and steroids....on propfol for sedation over days....leading to metabolic acidosis...renal failure etc.
 
Interesting....isnt it usually wtih long infusions of propofol...over 24 hrs?

Your patient had issues just with the induction dose?

Also, how did you diagnose it?
 
lol

Thought the private forum was for politcs and the public one for clinical.

If this is your plan ill just avoid reading your clinical scenarios.
 
I'm not interested in teaching nurses.


That is so CHILDISH.[/SIZE][/COLOR][/SIZE]

What is wrong with you?????? My 7 year old is more mature.

Our specialty is DOOMED....as long as you losers are our new residents...I'm glad it is doomed.

And I'm going to screw everyone one of you for as long as I can before I retire.

OVER and OUT.
 
You are pathetic. I never want someone like you representing me (or physicians in general). I wish I could be there when you learn the hard way, b/c son, its comin'.

I'm not interested in teaching nurses.
 
:laugh: relax dude. You are so uptight it's not even funny. I love it how you get all bent out of shape.
 
MMD's got a point.

I suppose you do too, not that I agree, but you started a clinical discussion and it'd be nice if you finished it here.
 
That's total BS. Why didn't you start your thread on the private forum and instead chose to waste our time here?

BTW, the reason I haven't joined the private forum is that I'm not going to provide some unknown wanker with my ASA ID which also happens to be my password to the ASA website(as is for everyone else). I caution everyone to be careful with such private information. For all I know, you could all well be CRNA's pretending to be MD's.
 
That's total BS. Why didn't you start your thread on the private forum and instead chose to waste our time here?

BTW, the reason I haven't joined the private forum is that I'm not going to provide some unknown wanker with my ASA ID which also happens to be my password to the ASA website(as is for everyone else). I caution everyone to be careful with such private information. For all I know, you could all well be CRNA's pretending to be MD's.

I may be a lot of things but BLADE is NO CRNA and neither is Toughlife.
The guy is for real and he means well. Your precious ASA number will be protected for whatever that is really worth. JPP, Noyac, Mil MD, Plankton, etc. and many others are members.

Tough, you need to FINISH this case here because you started it here.

Blade
 
Be a man, Tough, and continue the good discussion you started.


When the patient initially developed the symptoms she was taken to the ICU and, of course, sedated with propofol.

Initial workup included brain MRI which was normal. Labs showed elevated lactic acid and given her prior complaint of weakness neurology was consulted.

They recommended full work up which included blood ammonia, uric acid, alanine levels, TSH, CK, very long chain fatty acid analysis, karyotype and EMG with muscle biopsy. Full rheumatology panel sent as well.

All labs including rheum panel were negative. Alanine levels were noted to be elevated.

- Muscle biopsy with normal oxidative phosphorylation, EM/histo, but abnormal ETC (electron transport chain) testing at Complex 3.


We know know that propofol acts as an inhibitor of the electron transport chain complex 1 and there's literature that supports the notion the idea that it also affects the ATP synthase and UCPs (uncoupling proteins) that create the proton gradient that drives the phosphorylation of ATP and hence produce energy. These depressants effects are also seen in cardiac and brain mitochondria.

Volatile agents like halo, iso and sevo also have similar effect on the same complex albeit at different rates --20% for halo and iso and 10% for sevo. However, the fact that this patient had a congenitally deficient complex 3, and if you add to that the supressive actions that propofol and volatiles create on complex 1, you were seriously inhibiting her oxidative phosphorylation capacity.
 
Wow. Really impressive. I'm glad she was in a "real" hospital where the work-up could be done. Somewhere else it would be like "She is f-ed, end of story".

I hope your report gets accepted for publication.
 
familial hypokalemic periodic paralysis

This is kind of what I would have said, but I would have guessed hyperkalemic periodic paralysis.

My question is - why did you choose the hypokalemic variant of the myotonias? Why not hyperkalemic, or myotonia congenita? I have been trying to figure out a difference between these myotonias and how I will sort them out on a question, but can't seem to get my tiny brain around it.

Any thoughts?
 
Thanks for finishing the drug mystery. Genetic differences in drug metabolism & kinetics is becoming a big field of research. Someday we might be able to test for this stuff so you can make the best choice of drug to give rather than figure it out after.

I'll be looking forward to reading your article!
 
Thanks for finishing the drug mystery. Genetic differences in drug metabolism & kinetics is becoming a big field of research. Someday we might be able to test for this stuff so you can make the best choice of drug to give rather than figure it out after.

I'll be looking forward to reading your article!

Its all part of the Human Genome Project. We will know everyones genetic makeup and there will be absolutely no guessing. Life will be great, or will it?
 
Its all part of the Human Genome Project. We will know everyones genetic makeup and there will be absolutely no guessing. Life will be great, or will it?

hmmm - not so sure. But, let me finish my bottle of wine & I might be able to come up with an opinion:p.

But - if this were my 23 yo daughter (which, btw...I wish I still had - she's older than that:oops:) who had already undergone 3 surgeries & was due for an ablation - um, yeah, speaking only as a mom & not as a pharmacist - I'd sure want you to know as much as you can so you can choose & this situation wouldn't occur. Thats when your professional judgement comes into play - as in Blade's etomidate case....as the physician, you weigh the risks & benefits as it applies to the clinical situation presented. As a patient & parent & professional, thats why we trust you (& others...me, in my job) to weigh the choices & make the best decision possible under the circumstances presented. There will never be a "menu" of options one can choose - just more information to help you make that best judgement possible.

But...this is mixing personal & professional stuff. As a pharmacist - I'm very interested in pharmacogenetics & I'm of the opinion (opinion only & of no more value than the bandwidth it takes to send this!) we shouldn't interfere with medical progress just because it can be ill applied (I've also got an 88yo mom whom I'd never let be put in the position of your last case...). There are plenty of "guesses" still out there, but if we can minimize some of them - great! I'm old enough to remember stocking ether in the pharmacy. We've come a long way since then - progress!

My interest in pharmacogenetics began when Gibson & Reidenberg discovered in 1975 there were "fast" acetylators of procainamide which showed genetic variation. The Human Genome Project did not start formally until 1990. Funny - Gibson was a professor of mine.....:D So, this is not part of the Human Genome Project - just an extension of drug development, which has definitely been influenced by the information which has been discerned from the project.

So - yeah - choices..professional & personal - difficult choices on all sides, but still - educationally challenging when they occur, at least to me & I hope toughlife gets it published!

I appreciate & respect your comment & many share the concern & feel we shouldn't go where we are going. I just don't agree. Both are valid arguments worthy of respect - thus...the ethics of our professions.
 
hmmm - not so sure. But, let me finish my bottle of wine & I might be able to come up with an opinion:p.

But - if this were my 23 yo daughter (which, btw...I wish I still had - she's older than that:oops:) who had already undergone 3 surgeries & was due for an ablation - um, yeah, speaking only as a mom & not as a pharmacist - I'd sure want you to know as much as you can so you can choose & this situation wouldn't occur. Thats when your professional judgement comes into play - as in Blade's etomidate case....as the physician, you weigh the risks & benefits as it applies to the clinical situation presented. As a patient & parent & professional, thats why we trust you (& others...me, in my job) to weigh the choices & make the best decision possible under the circumstances presented. There will never be a "menu" of options one can choose - just more information to help you make that best judgement possible.

But...this is mixing personal & professional stuff. As a pharmacist - I'm very interested in pharmacogenetics & I'm of the opinion (opinion only & of no more value than the bandwidth it takes to send this!) we shouldn't interfere with medical progress just because it can be ill applied (I've also got an 88yo mom whom I'd never let be put in the position of your last case...). There are plenty of "guesses" still out there, but if we can minimize some of them - great! I'm old enough to remember stocking ether in the pharmacy. We've come a long way since then - progress!

My interest in pharmacogenetics began when Gibson & Reidenberg discovered in 1975 there were "fast" acetylators of procainamide which showed genetic variation. The Human Genome Project did not start formally until 1990. Funny - Gibson was a professor of mine.....:D So, this is not part of the Human Genome Project - just an extension of drug development, which has definitely been influenced by the information which has been discerned from the project.

So - yeah - choices..professional & personal - difficult choices on all sides, but still - educationally challenging when they occur, at least to me & I hope toughlife gets it published!

I appreciate & respect your comment & many share the concern & feel we shouldn't go where we are going. I just don't agree. Both are valid arguments worthy of respect - thus...the ethics of our professions.

SDN1977 provided a corollary superior to what I'm capable of.

SO HERE'S JET, THE BOARD CERTIFIED, COPENHAGEN DIPPIN', NINE-INCH-LIFTED-TRUCK-DRIVIN'S OPINION:

Weigh risks and benefits.

Ventricular arrythmias when you are young is some very bad s h it.

So you need to undergo an operation to make it better.

Because of your genetic makeup, said sequalae of said operation is pretty gruesome....BUT IT AIN'T GONNA KILL YA. ITS GONNA MAKE YOU UNCOMFORTABLE FOR A FINITE PERIOD. BUT YOURE GONNA BE ALRIGHT.

ON THE OTHER HAND, THAT VENTRICULAR ARRYTHMIA S H IT YOU'VE GOT MAY KILL YOU, IF IT PROCEEDS UNADDRESSED.


So yeah, lets mentally masturbate LIKE FLEAS why the patient has unexplained weakness after operations.....aberrant mitochondria, blah blah blah....

OR, lets fix the problem and deal with the after-effects when its done, which are not life-threatening.

Yeah, I hear the academic hecklers.

But what I'm concerned about is the END RESULT....

who was the trauma surgeon from Texas that had a TV show follow him for a period of time? That dude knew the REAL DEAL.

screw all the mental masturbation about why something is happening.

LETS FIX IT NOW.

AND FIGURE IT OUT LATER.
:thumbup:
 
SDN1977 provided a corollary superior to what I'm capable of.

SO HERE'S JET, THE BOARD CERTIFIED, COPENHAGEN DIPPIN', NINE-INCH-LIFTED-TRUCK-DRIVIN'S OPINION:

Weigh risks and benefits.

Ventricular arrythmias when you are young is some very bad s h it.

So you need to undergo an operation to make it better.

Because of your genetic makeup, said sequalae of said operation is pretty gruesome....BUT IT AIN'T GONNA KILL YA. ITS GONNA MAKE YOU UNCOMFORTABLE FOR A FINITE PERIOD. BUT YOURE GONNA BE ALRIGHT.

ON THE OTHER HAND, THAT VENTRICULAR ARRYTHMIA S H IT YOU'VE GOT MAY KILL YOU, IF IT PROCEEDS UNADDRESSED.


So yeah, lets mentally masturbate LIKE FLEAS why the patient has unexplained weakness after operations.....aberrant mitochondria, blah blah blah....

OR, lets fix the problem and deal with the after-effects when its done, which are not life-threatening.

Yeah, I hear the academic hecklers.

But what I'm concerned about is the END RESULT....

who was the trauma surgeon from Texas that had a TV show follow him for a period of time? That dude knew the REAL DEAL.

screw all the mental masturbation about why something is happening.

LETS FIX IT NOW.

AND FIGURE IT OUT LATER.
:thumbup:

I hear you loud and clear but the issue is that she was going to undergo radiofrequencly ablation for the arrythmias induced by the anesthetics. So how do you propose we 'fix' the arrythmia?
I know as an anesthesiologist you don't care about what happens beyond the PACU , but if you were the SICU anesthesia attending where this patient ends up?

I think we in anesthesia take such a minimalist approach that we really look like nothing more than mere technicians. If we want to change our image, we need to get away from that mentality. we are physicians first and anesthesiologist second, so there's nothing wrong with thinking like one.
 
So speaking of mitochondrial mutations...

We had our weekly grand rounds this morning and guess who are speakers were? Philip Morgan and Margaret Sedensky. Their primary research is in genetic mechanisms of anesthetic effects. They work mostly on worms, but they have identified several genes that can substantially alter the effects of anesthetics. Like knock this gene out and now the worms are out at 1/10 the concentration of halothane that it takes to provide anesthesia for a wild type worm.

They did offer one interesting clinical tid bit. At Case Western, where they work, one of the other researchers is big into mitochondrial diseases. He freqently sends his clinic pateints (mostly kids) in for muscle biopsies, which are done under GA. So, started collecting data on these kids. They would titrate sevo to BIS less than 60. Once the muscle biopsy was done they looked at some of the genes the research in their lab, and compared volatile response in kids with similar mutations to their worms. Sure enough they had a kid who had his BIS hit the floor with the sevo a super low levels. His mutation they found out later matched up with one of theirs.

Anyway, neat side bar.
 
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