Safety of isoflurane and epidural anesthesia in a patient with hereditary coproporphyria

Abstract

Porphyrias are inherited or acquired disturbances in heme synthesis, and are usually divided into two main groups, erythropoietic and hepatic, according to the two major sites of heine synthesis [1]. Among hepatic porphyrias, acute intermittent porphyria (AIP), variegate porphyria (VP), and hereditary coproporphyria (HCP) may give rise to an acute attack which presents with mild to severe abdominal and neuropsychiatric symptoms in response to stress, infection, fasting/dehydration, endogenous hormonal fluctuations, excessive alcohol intake, and certain drugs [2-4]. HCP is an autosomal dominant hepatic porphyria resulting in a deficiency of the coproporphyrinogen oxidase enzyme. The incidence of HPC is uncertain, since most patients are asymptomatic. Acute attacks are indistinguishable from those in AIP and VP and are precipitated by the same factors. During acute attacks, increased urinary excretion of f>-aminolevulinic acid (ALA) and porphobilinogen (PBG) occurs in all groups, but the patterns of porphyrins in urine and feces differ. Increased fecal excretion of coproporphyrinogen III is characteristic [2]. In the anesthetic management of such patients with porphyria, the selection of appropriate anesthetic agents and techniques is important. Although a variety of drugs have been implicated in precipitating an acute attack [5], it is difficult to predict which drugs will be porphyrogenic. There have been

DOI: 10.1007/BF02482076

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Cite this paper

@article{Yamamori1996SafetyOI, title={Safety of isoflurane and epidural anesthesia in a patient with hereditary coproporphyria}, author={Yuji Yamamori and Mariko Sumi and Manami Yamanaka and Yoshihiro Kosaka}, journal={Journal of Anesthesia}, year={1996}, volume={10}, pages={80-82} }