hospital with status epilepticus. She was treated with a propofol infusion at
5mg/kg/hr which was maintained due to perceived continuing seizure activity. On hospital day 5, she developed an acute metabolic deterioration –
rhabdomyolysis : CPK 100,000, AG metabolic acidosis (HCO3 18) and non-oliguric
AKI. Her EKG also became abnormal (RBBB). The diagnosis was propofol infusion
biochemical manifestations. Based
on 83 case reports from 1992-2007, PRIS
is characterized by:
sustained VTs, heart failure, or bradycardia)
requirement for the number of clinical manifestations a patient must exhibit to
meet diagnosis for PRIS, a prospective study showed that most patients exhibit
at least 3 defining manifestations within 3 days of propofol use. In the same study, the
incidence of PRIS was found to be a low 1% –similar to other estimates. However, PRIS is associated with high
mortality, up to 30% in some studies. Moreover,
because many of these manifestations are common, the presence of any of them
could be attributed to another etiology, thus delaying diagnosis of PRIS. For instance, in the case vignette, the
initial rise of CK was attributed to seizure rather than PRIS.
chain/ impairs oxygen utilization. Propofol or its metabolites inhibits fatty-acid oxidation leading to buildup
of toxic fatty acid intermediates. As described in this nice review.
than 4 mg/kg/hr —the usual adult maintenance dose is 0.3 to 3mg/kg/hr; duration
of Propofol use greater than 24hr; exogenous cathecholamines and
corticosteroids; poor intake of carbohydrate, see this reference.
Early recognition/diagnosis; Cessation of propofol infusion; Cardiopulmonary
support; Hemodialysis (strongly advocated by expert opinion and outcome of case
However, there are no known RCT of use of renal replacement therapy in
the treatment of PRIS. Nonetheless, in
case series, survivors of PRIS are more likely to have received HD/CVVH. Therefore, prolonged use of HD/CVVH is worth
considering by renal consult service.
Because propofol is lipophilic and has volume of distribution of 20-40L,
it is poorly cleared by HD/CVVH, prolonged RRT is likely needed for PRIS
transitioned to intermittent HD. At the
time of her discharge, she was off HD with her Cr back to baseline.