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Case Report

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Perioperative management of porphyria: a case report

  • ROBERT BARONICA1
  • KLAUDIJA PRLIĆ1
  • MARTINA ČALUŠIĆ1
  • MARIJA PLANINIĆ1
  • ANTE PENAVIĆ1
  • MARINA KLJAKOVIĆ GAŠPIĆ1
  • KORALJKA BAČIĆ BARONICA2

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

*Corresponding Author(s): ROBERT BARONICA E-mail: rbaronica@gmail.com

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Abstract


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. 

Key words

porphyria, perioperative man-agement, hemodynamic instability

Cite And Share

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.

References

1. Anderson KE, Sassa SS, Bishop DF, Desnick RJ. Disorders of heme biosynthesis: X-linked sideroblastic anemia and the porphyrias. In: Scriver CR, Beaudet AL, Sly WS, Valle, D, Eds. The metabolic basis of inherited disease, 8th edn. New York: McGraw-Hill, 2001.p.453-462.

2. Puy H, Gouya L, Deybach JC. Porphyrias. Lancet 2010;375(9718):924-937.

3. Allman KG, Wilson IH. Oxford handbook of anaesthesia 3rd ed. New York: Oxford University Press;2011.

4. James MFM, Hift RJ. Porphyrias. Br J Anaesth 2000; 85:143-153.

5. Andersson C, Bjersing L, Lithner F. The epidemiology of hepatocellular carcinoma in patients with acute intermittent porphyria. J Intern Med 1996;240(4):195-201.

6. Thadani H, Deacon A, Peters T. Diagnosis and management of porphyria. BMJ 2000;320(7250):1647-1651.

7. Rigal JC, Blanloeil Y. Anaesthesia and porphyria. Minerva Anestesiol 2002;68(5):326-331.

8. Balwani M, Desnick RJ. The porphyrias: advances in diagnosis and treatment. Blood 2012;120(23):4496-4504.

9. Handschin C, Lin J, Rhee J, Peyer AK, Chin S, Wu PH, Meyer UA, Spiegelman BM. Nutritional regulation of hepatic heme biosyn-thesis and porphyria through PGC-1alpha. Cell 2005;122(4):505-515.

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