Abstract:
:The case of a woman with protoporphyria who developed liver failure and underwent liver transplantation is described. During the pretransplant episode of liver failure she developed quadriparesis that rapidly progressed after transplantation to a severe polyneuropathy. Following transplantation she also developed a second-degree burn of the light-exposed abdominal wall. The neuropathy resembled that observed in other forms of porphyria, and it is proposed that the extreme disturbance of protoporphyrin levels associated with protoporphyrin-induced liver failure caused this neuropathy. Such a neuropathy has not previously been described in protoporphyria. Erythrocyte protoporphyrin levels remain high and fecal levels normal, although results of liver tests are normal. She remains photosensitive, which emphasizes that although liver transplantation may be lifesaving in this disorder, it is not curative, and care must be taken to prevent photosensitive damage to skin and light-exposed internal organs.
journal_name
Gastroenterologyjournal_title
Gastroenterologyauthors
Herbert A,Corbin D,Williams A,Thompson D,Buckels J,Elias Edoi
10.1016/0016-5085(91)90680-jsubject
Has Abstractpub_date
1991-06-01 00:00:00pages
1753-7issue
6eissn
0016-5085issn
1528-0012pii
0016-5085(91)90680-Jjournal_volume
100pub_type
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