Nephrotic syndrome

[militarymd]

Case consulted on yesterday.

40 something guy coming down for ex-lap because of perforated viscous.

Presumed cause of perforation...several week course of high dose steroids for treatment of fsg with nephrotic syndrome.

Clinical presentation....abdominal pain with guarding...free air in imaging study. Worsening tachypnea....Severe edema up to umbilicus secondary to nephrotic syndrome....progressive tachycardia....blood pressure currently stable....130/80.

Laboratory findings....HCO3 22...chloride 115....so mild acidosis....hct 27..plt 16...cr 2.5...pt/ptt/inr normal...those are the pertinent positives...

Any thoughts...especially from the knowledgeable attendings.

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[Pilot Doc]

Any thoughts...especially from the knowledgeable attendings.

That's not me ... but can you flesh out the question? Are you looking for how to optimize before urgent/emergent surgery? Actual anesthetic technique?

 

[militarymd]

That's not me ... but can you flesh out the question? Are you looking for how to optimize before urgent/emergent surgery? Actual anesthetic technique?

The patient obviously is headed to the OR....you can't cancel it...so I was thinking in terms of:

1) risks unique to this patient
2) interventions that may decrease the risk

 

[VentdependenT]

ASA 4E

Not much you can do about the FSG eh.

Slug the dude with a big whopper of a dose of solumedrol: 1g. Preop.

protein restriction: no albumin

?use enalaprit for hypertension? I don't have any experience from this drug but my crappy book here says that an ace-i "may improve glomerular flow dynamics." WHo the hell knows. This stuff takes 10-20 min to kick in and hangs out fer a while.

"avoid hypotension"

Get the platelets up to 50k

uhhh....transfuse if he dips below 24. thats my trigger and I'm sticken to it. If one wanted to give him a unit before hand to maximize blood oxygen content while keeping viscosity in check it'd be ok in my little book.

Sounds like a setup for ARDS or some unholy immunesystem explosion (DIC, MSOF, gut ischemia)

 

[SexPanther]

protein restriction: no albumin

Vent,

Could you clarify why we wouldn't give albumin to a pt. suffering from nephrotic syndrome? It's not making sense to me since by definition they have hypoalbuminemia.

Although I'm just a student, I think some other things to consider in a pt. w/CKD/FSGS/nephrotic syndrome are electrolytes/arrhythmias, volume overload, and hypercoagulability 2/2 to loss of protein C in the urine seen w/nephrotic syndrome.

 

[militarymd]

Vent,

Could you clarify why we wouldn't give albumin to a pt. suffering from nephrotic syndrome? It's not making sense to me since by definition they have hypoalbuminemia.

Although I'm just a student, I think some other things to consider in a pt. w/CKD/FSGS/nephrotic syndrome are electrolytes/arrhythmias, volume overload, and hypercoagulability 2/2 to loss of protein C in the urine seen w/nephrotic syndrome.

Protein C....? not Antithrombin III???

 

[SexPanther]

Protein C....? not Antithrombin III???

Yeah, you're right, protein C/S's activity is altered in nephrotic syndrome while AT-III is lost in the urine.

So other than the brain fart above, are those valid concerns? Appreciate the input.

 

[zippy2u]

Knowledgeable attending I'm not but let's give it a whirl. 1.) Risks to patient. Go up to pts room and gather around the 20 something relatives and have a "kumbaha" gathering and tell the pt he may die. Make sure some of the relatives hear this. Use to know some gas docs who were of the "holy roller" persuasion and hold hands and say some fancy prayers but that would not be me. Liked workin' in those Catholic hospitals with cases like these 'cause I'd call the nuns up to the room to get the kumbaha thing going in high gear. Once you tell him and the family he may die the other risks won't be heard or remembered. 2.) Interventions to possibly improve outcome: Since you have all these ICU skills/ fellowship you do the case or direct the CRNA closely. Give 2 units of PRBCs and 10 units of platelets perioperatively. Throw a central line (via cordis) and A-line in and monitor pressures. Make sure the surgeon is a seasoned pro and not a rookie. Keep pt. intubated and tell surgeon you'll manage all the "flea" details to keep the guy in optimal condition. Surgeon will love this and you'll earn extra brownie points. Regards, ---Zip

 

[jetproppilot]

Case consulted on yesterday.

40 something guy coming down for ex-lap because of perforated viscous.

Presumed cause of perforation...several week course of high dose steroids for treatment of fsg with nephrotic syndrome.

Clinical presentation....abdominal pain with guarding...free air in imaging study. Worsening tachypnea....Severe edema up to umbilicus secondary to nephrotic syndrome....progressive tachycardia....blood pressure currently stable....130/80.

Laboratory findings....HCO3 22...chloride 115....so mild acidosis....hct 27..plt 16...cr 2.5...pt/ptt/inr normal...those are the pertinent positives...

Any thoughts...especially from the knowledgeable attendings.

Fluids, fluids, fluids.

Necessary for adequate perfusion during the case...even if he blows up like a balloon.

Blood products as necessary...starting with platelets, prbcs like everyone mentioned...anticipate more-than-usual bleeding

Would go with two large bore peripheral IVs rather than risk a problem with a central line from thrombocytopenia...

A line...

correct acidosis if it gets worse....

keep him peeing and warm.

Anticipate keeping the tube in post op.

 

[Noyac]

Mostly I'd want the symptoms of Nephrotic synd treated like pulmonary edema, ascites, low plts (in this case), etc.

In this case, you may not have time to treat all these things. You will need to proceed. They are usually hypercoaguable with the loss of clotting proteins. Not here, with plts at 16K. Spontaneous bleeding occurs at <20K, and surgical bleeding is usually well controlled if >50K. Treat as indicated.

Continue fluids as necessary to maintain pressure. I'm assuming acidosis is fluid related and it should correct with fluids or PRBC's (if necesary, not currently as I see it).

 

[militarymd]

This things that I was concerned about...ie things that are going to kill him.

1) bleeding to death in the OR

2) pulmonary embolism sometime later in the hospital course because of his hypercoagulable state...and for being critically ill....yes ...even in light of a platelet count of 16.

My recommended Plan:

1 platelets for obvious reasons....although patient had no clinical evidence of bleeding when we were sticking him.

2 FFP...to replace anti-thrombin III......so that the lovenox will be an effective thromboembolism prophylaxis prior to start of surgery.

3 Non-pharmacologic pro-coagulant therapy...ddavp and amicar to hopefully decrease bleeding in the perioperative period.

I believe the anesthesia team only carried out 1 and 3.....patient did fine....for now.....we will have to wait and see if he makes it home.....something that I would say most anesthesia providers don't follow up on.

 

[jetproppilot]

This things that I was concerned about...ie things that are going to kill him.

1) bleeding to death in the OR

2) pulmonary embolism sometime later in the hospital course because of his hypercoagulable state...and for being critically ill....yes ...even in light of a platelet count of 16.

My recommended Plan:

1 platelets for obvious reasons....although patient had no clinical evidence of bleeding when we were sticking him.

2 FFP...to replace anti-thrombin III......so that the lovenox will be an effective thromboembolism prophylaxis prior to start of surgery.

3 Non-pharmacologic pro-coagulant therapy...ddavp and amicar to hopefully decrease bleeding in the perioperative period.

I believe the anesthesia team only carried out 1 and 3.....patient did fine....for now.....we will have to wait and see if he makes it home.....something that I would say most anesthesia providers don't follow up on.

Could (non pharmacologic) pro-coagulant therapy increase the risk of PE?

 

[proman]

NEJM had a nice review of nephrotic syndrome. A little dated (1998) but short and to the point. It's in the 4/23/98 issue by Orth and Ritz.

 

[militarymd]

Could (non pharmacologic) pro-coagulant therapy increase the risk of PE?

No way to know...that's why I had recommended both procoagulant and anti-coagulant therapies.

 

[TIVA]

Propofol, LMA, full speed ahead

 

[Noyac]

Propofol, LMA, full speed ahead

 

[dhb]

Propofol, LMA, full speed ahead

 

[militarymd]

Propofol, LMA, full speed ahead

That certainly resembles a technique that I favor.

 

[Planktonmd]

Case consulted on yesterday.

40 something guy coming down for ex-lap because of perforated viscous.

Presumed cause of perforation...several week course of high dose steroids for treatment of fsg with nephrotic syndrome.

Clinical presentation....abdominal pain with guarding...free air in imaging study. Worsening tachypnea....Severe edema up to umbilicus secondary to nephrotic syndrome....progressive tachycardia....blood pressure currently stable....130/80.

Laboratory findings....HCO3 22...chloride 115....so mild acidosis....hct 27..plt 16...cr 2.5...pt/ptt/inr normal...those are the pertinent positives...

Any thoughts...especially from the knowledgeable attendings.

Here is how i would do that the private practice waynot a knowledgeable attending)
A line in holding, go to OR, RSI and ETT, Central Line, Monitor CVP and urine output, Replace Fluids accordingly, keep intubated postop, Next patient!