Joanna K. Dowman, MBChB(Hons); Bridget K. Gunson; Simon Bramhall, MD; Mike N. Badminton, MBChB, PhD; Philip N. Newsome, PhD
Potential Conflicts of Interest: Dr. Dowman: Grant received: Wellcome Trust. Grant received (money to institution): Wellcome Trust. Grant pending: Wellcome Trust.
Dowman J., Gunson B., Bramhall S., Badminton M., Newsome P.; Liver Transplantation From Donors With Acute Intermittent Porphyria. Ann Intern Med. 2011;154:571-572. doi: 10.7326/0003-4819-154-8-201104190-00015
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Published: Ann Intern Med. 2011;154(8):571-572.
This article has been corrected. For original version, click "Original Version (PDF)" in column 2.
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).
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