Use of Regional Anaesthesia in a Patient With Acute Porphyria

McNeill, M. J.; Bennet, A.

doi:10.1093/bja/64.3.371

Cited by 30 publications

(1 citation statement)

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“…Regionalanästhesien bei Porphyriepatienten zu vermeiden seien (36), insbesondere da bei perioperativer Verschlimmerung der Neuropathie mit juristischen Bedenken zu rechnen sei. ~r f a h m n~e n arn eigenen Patientengut bestätigen die Aussagen der neueren Literatur (11,37,46), daß Reeionalanästhesien bei diesen Patienten ~roblemlos durchgeWhhrt werden können. lppen und F~~~S hatten bereits 1984 in ihrem Übersichtsartikel 33 Dermatologische Erkrankungen mit Blasenbildungen und extremer Hautfragilität wie z.…”

Section: Beurteilung Anästhesierelevanter Substanzen Barbiturateunclassified

Anästhesie und akute hepatische Porphyrien

Böhrer

Schmidt²,

Martín³

1992

Anästhesiol Intensivmed Notfallmed Schmerzther

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Section: Beurteilung Anästhesierelevanter Substanzen Barbiturateunclassified

Anästhesie und akute hepatische Porphyrien

Böhrer

Schmidt²,

Martín³

1992

Anästhesiol Intensivmed Notfallmed Schmerzther

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Liver transplantation for porphyria: Who, when, and how?

et al. 2007

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Porphyrias are a heterogenous group of diseases that may result in disabling or life threatening neurovisceral symptoms and/or cutaneous photosensitivity. In acute intermittent porphyria, the clinical features, particularly neurological symptoms, may be life-threatening and disabling. Conventional treatment with human hemin, though effective in reducing symptoms, does not reverse neuropathy when structural nerve damage has occurred and may cause intense phlebitis. Liver transplantation (LT) may be considered as treatment for those with repeated life-threatening acute attacks resulting in poor quality of life, requirement of ventilatory support, and progressive loss of venous access due to hemin infusion. Patients with variegate porphyria (VP) present after puberty with neurovisceral symptoms and skin manifestations. LT resolved VP in the 1 patient reported in the literature. Aminolaevulinic acid dehydratase deficient porphyria is a rare autosomal recessive disorder and a child who presented with failure to thrive and required transfusions and parenteral nutrition did not improve with LT. In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin in the bone marrow. Protoporphyrin is hepatotoxic and pigment loading of hepatocytes and bile canalicular sludging may result in progressive cholestasis and cirrhosis. LT is beneficial for such patients with end-stage liver disease. Perioperative management includes use of filters on operative lights to prevent skin burns and intestinal perforation. Other concerns include development of neuropathy, biliary complications, and recurrent liver disease. This review addresses the rationale, patient selection, evaluation, management issues, and technique of performing LT in various types of porphyria.

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