Erythropoietic protoporphyria: unusual skin and neurological problems after liver transplantation.

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

Gastroenterology

journal_title

Gastroenterology

authors

Herbert A,Corbin D,Williams A,Thompson D,Buckels J,Elias E

doi

10.1016/0016-5085(91)90680-j

subject

Has Abstract

pub_date

1991-06-01 00:00:00

pages

1753-7

issue

6

eissn

0016-5085

issn

1528-0012

pii

0016-5085(91)90680-J

journal_volume

100

pub_type

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