Perioperative management of porphyria: a case report
1University Department of Anaesthesiology, Reanimatology and Intensive Care, University Hospital Centre Zagreb
2University Department of Neurology, University Hospital Sveti Duh, Zagreb
DOI: 10.22514/SV101.062015.18 Vol.10,Issue S1,June 2015 pp.63-65
Published: 22 June 2015
The porphyrias are rare inherited meta-bolic disorders of the heme biosynthesis pathway. Acute intermittent porphyria is the most common form that may result in acute porphyric crises with abdominal pain, vomiting, hemodynamic distur-bances, autonomic dysfunction, pyrexia and neurological deterioration. Provoca-tive factors include hormonal fluctuations, fasting, dehydration, smoking, excessive alcohol or illegal drugs intake and stress from illness or surgery. However, the most frequent triggers are cytochrome P450-in-ducing drugs, especially in relation to an-aesthesia. We report a case of a 63-year-old female with acute intermittent porphyria and severe hemodynamic instability dur-ing and after liver resection taken for hepa-tocellular carcinoma. The procedure was predominantly characterized by unusual hemodynamic instability with refractory hypertension, despite adequate analgesia and depth of anaesthesia. Several differ-ent treatments failed to reduce high blood pressure. There is a possibility that some drugs used in the perioperative period caused acute porphyric crises, which was manifested by severe hemodynamic insta-bility. Autonomic neuropathy might have caused labile blood pressure as well. A pre-cise etiology of hemodynamic instability in the presented case is difficult to assess, since other provocative factors like fasting, dehydration and stress from surgery were also present. A careful anaesthetic plan and treatment and postoperative surveil-lance in the ICU are cornerstones in the management of patients with porphyria subjected to major surgical procedures.
porphyria, perioperative man-agement, hemodynamic instability
ROBERT BARONICA,KLAUDIJA PRLIĆ,MARTINA ČALUŠIĆ,MARIJA PLANINIĆ,ANTE PENAVIĆ,MARINA KLJAKOVIĆ GAŠPIĆ,KORALJKA BAČIĆ BARONICA. Perioperative management of porphyria: a case report. Signa Vitae. 2015. 10(S1);63-65.
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