Erythropoietic protoporphyria: unusual skin and neurological problems after liver transplantation.
Gastroenterology
; 100(6): 1753-7, 1991 Jun.
Article
in En
| MEDLINE
| ID: mdl-2019380
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.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Photosensitivity Disorders
/
Polyneuropathies
/
Porphyrias
/
Postoperative Complications
/
Liver Transplantation
/
Liver Diseases
Type of study:
Etiology_studies
Limits:
Adult
/
Female
/
Humans
Language:
En
Journal:
Gastroenterology
Year:
1991
Type:
Article
Affiliation country:
United kingdom