Liver transplantation for erythropoietic protoporphyria. Report of a new case with subsequent medium-term follow-up.
We report a new case of successful liver transplantation in a 36-year-old patient with terminal hepatic failure due to erythropoietic protoporphyria. Data regarding protoporphyrin levels in erythrocytes and feces, before and after transplantation, seem to indicate that in this case protoporphyrin overproduction was in part due to liver synthesis. Four years after surgery, the patient is completely free of skin photosensitivity. His liver function tests are normal; there are no visible protoporphyrin deposits or ultrastructural abnormalities in his new liver. However, recurrence of the disease in the long term cannot be excluded, since erythrocyte protoporphyrin levels remained elevated after liver transplantation.
Mion
F B
FB
Department of Digestive Diseases, Hôpital Edouard Herriot, Lyon, France.
Faure
J L
JL
Berger
F
F
McGregor
B
B
Perrot
H
H
Paliard
P
P
D000328:Adult; D017454:Dermatitis, Photoallergic / Q000209:etiology; D005500:Follow-Up Studies; D006801:Humans; D008099:Liver / Q000378:metabolism; D016031:Liver Transplantation; D008297:Male; D017121:Porphyria, Hepatoerythropoietic / Q000150:complications / Q000378:metabolism / Q000601:surgery*; D011524:Protoporphyrins / Q000096:biosynthesis; D012008:Recurrence
D011524:Protoporphyrins