Skuteczna resusctytacja i powrót chorego do aktywności życiowej po zatrzymaniu krążenia w przebiegu nierozpoznanej porfirii – opis przypadku

Agnieszka Pikulska¹, Marcin Błach¹, Ewa Marzec-Lewenstein¹, Stanisław Kępiński¹, Arystarch Makowski¹, Danuta Nocoń², Anita Gregor³, Józef Kładny⁴, Romuald Bohatyrewicz¹

Summary
Background. Porphyrias belong to group of metabolic diseases. Due to their rare occurrence (8:100 000 in Poland) they may not be recognized immediately in case of severe abdominal pain. Case report. A 38-year-old male patient was admitted to the ICU after cardiac arrest that occurred in the neurologic department, where he was transferred after being treated in a regional hospital for abdominal pain and renal failure. He underwent haemodialysis and emergency laparotomy (thiopentone was used for induction). On admission to the neurologic department he was confused, hypertensive and quadriparetic. The preliminary diagnosis of the acute intermittent porphyria was made and propranolol/glucose treatment was started. Despite that, the patient´s condition worsened and cardiac arrest occurred. He was successfully resuscitated and transferred to the ICU, where haem substitution was started. During following days the man became septic and suffered further porphyric crises; he was ventilated for 36 days and eventually recovered. After 56 days of the ICU stay he was transferred to the revalidation department and after next 8 months recovered sufficiently to return to his prehospital life activities. Discussion and conclusions. Because of variety of symptoms, acute intermittent porphyria may be difficult to recognize. In the described case, the cause of abdominal pains was not recognized in regional hospital and institution of a proper treatment was delayed resulting in a near-fatal accident. We conclude that acute intermittent porphyria should be always in the back of mind of an anaesthetist who is facing an emergency abdominal procedure. In any case of suspicion, triggering drugs should be avoided.

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