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Liver transplantation from donors with acute intermittent porphyria [Letter]

Dowman, Joanna K., Gunson, Bridget K., Bramhall, Simon, Badminton, Michael Norman and Newsome, Philip N. 2011. Liver transplantation from donors with acute intermittent porphyria [Letter]. Annals of International Medicine 154 (8) , pp. 571-572.

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Background: Acute intermittent porphyria (AIP) results from partial deficiency of the enzyme porphobilinogen deaminase (1). The clinical disease includes life-threatening neurovisceral attacks from neuronal damage. The cause of these attacks has not been established but involves the heme precursor δ-aminolevulinic acid (ALA) (2). The liver is the major source of excess ALA production, and transplanting a normal liver into a patient with AIP effectively resolves this disease (3). Because clinical expression of AIP is more common in premenopausal women, we hypothesized that a liver removed from a premenopausal woman who is receiving a normal liver allograft could safely be …

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Subjects: R Medicine > RD Surgery
Publisher: American College of Physicians
ISSN: 1539-3704
Last Modified: 04 Jun 2017 03:28

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