Anästhesie mit Propofol bei einem exazerbierten Verlauf der akuten intermittierenden Porphyrie

Abstract

In the eighth week of pregnancy the medical indication for induced abortion was established due to an exacerbating acute intermittent porphyria with life-threatening neurological symptoms. delta-aminolaevulinic acid and porphobilinogen were excessively increased in urine prior to the operation. Anaesthesia was induced with a bolus of propofol, alfentanil and droperidol, maintained by 67% of nitrous oxide and small bolus injections of the three drugs. The patient regained consciousness immediately after the operation, and reached full orientation and cooperativeness within five minutes. The postoperative period remained uneventful and the neurological and psychological symptoms returned to the pre-exacerbation status. Chorion gonadotropins and porphyria markers decreased within the next four weeks accompanied by a simultaneously progressing clinical improvement. The use of propofol and the other drugs appears justified even in exacerbated porphyria.

Uneventful propofol anaesthesia in a patient with acute intermittent porphyria

“…However, there is some evidence that propofol does not exert porphyrinogenic effects, when given to patients with clinical symptoms of a porphyric crisis or elevated urinary porphyrin levels. Propofol was used with apparent safety in three patients with AIP during a porphyric crisis: in two patients for induction and maintenance of anaesthesia [46, 47] and in a mechanically ventilated patient for long‐term sedation (32 days) [45]. No further deterioration was detected in the patients concerned neither in clinical symptoms, nor in the biochemical measurements related to haem biosyntheis.…”

“…evaluated in the study did not include propofol. One may therefore not conclude that ured post-operatively [40,49] AIP during a porphyric crisis: in two patients for in-Therefore, some authors consider it unsafe to use duction and maintenance of anaesthesia [46,47] and propofol as a continuous infusion with a total dose in a mechanically ventilated patient for long-term greater than 1000 mg [51]. The evidence patient with asymptomatic VP, who received a total available suggests that this anaesthetic agent may dose of propofol 900 mg for induction and mainhave clinically useful antioxidant properties [33][34][35] tivity to a particular drug may depend on whether or Nevertheless, in some of these cases elevated pornot the patient is in the latent or acute phase of the phyrin levels, compared with base levels, were measdisease [30].…”

Testing the porphyrinogenicity of propofol in a primed rat model

“…The literature contains several reports on the use of propofol in patients with acute hepatic porphyrias. With the exception of one small series of 13 patients from South Africa [23], these publications have usually appeared as single case reports [24,25] or as letters to the editor [26][27][28][29][30][31][32]. In general, propofol is considered to be a safe agent in acute porphyria.…”

Propofol zur Narkose und Kurzzeitsedierung

“…Propofol ist kein MH-Trigger wie in vitro-und in vivo-Studien belegen[37, 51]. Auch für den Einsatz von Propofol bei Patienten mit Porphyrie liegen positive Befunde vor[38, 56], es dürfte also problemlos bei Porphyrie einsetzbar sein.…”