Liver transplantation for porphyria: Who, when, and how?
Avnish Kumar Seth, Michael N. Badminton, Darius Mirza, Scott Russell, Elwyn Elias – 30 August 2007 – 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.