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Signa Vitae

Journal of Anaesthesia, Intensive Care and Emergency Medicine

Perioperative management of porphyria: a case report

Abstract

The porphyrias are rare inherited metabolic 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 disturbances, autonomic dysfunction, pyrexia and neurological deterioration. Provocative 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-inducing drugs, especially in relation to anaesthesia. We report a case of a 63-year-old female with acute intermittent porphyria and severe hemodynamic instability during and after liver resection taken for hepatocellular carcinoma. The procedure was predominantly characterized by unusual hemodynamic instability with refractory hypertension, despite adequate analgesia and depth of anaesthesia. Several different 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 instability. Autonomic neuropathy might have caused labile blood pressure as well. A precise 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 surveillance in the ICU are cornerstones in the management of patients with porphyria subjected to major surgical procedures.

Key words: porphyria, perioperative management, hemodynamic instability

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Porphyrias – diagnostic challenge in Emergency room

Abstract

Porphyrias form a heterogeneous group of disorders of haem biosynthesis. They are often missed or wrongly diagnosed in the emergency department. Acute porphyrias present most commonly as abdominal pain or as neurological or atypical psychiatric symptoms. The variety of clinical features may delay diagnosis, and unrecognized disease is potentially life-threatening. The frequency and severity of attacks vary widely. In some people, this disease remains latent throughout life, even in the presence of precipitating factors. Other people experience frequent and sometimes life-threatening attacks even in the apparent absence of exogenous precipitating factors.

Laboratory diagnosis of acute porphyria includes porphyrin precursors delta aminolevulic acid and porphobilinogen. Patients presenting with acute crises often have several-fold increases in PBG above the reference interval, usually > 10 times the upper reference limit. Hyponatremia and rabdomyolysis are relatively common manifestation accompanying acute attack.

Key words: porphyria, laboratory diagnostic, emergency room

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