[Liver disease in erythropoietic protoporphyria]. / Leversygdom ved erytropoietisk protoporfyri.
Ugeskr Laeger
; 155(10): 697-700, 1993 Mar 08.
Article
en Da
| MEDLINE
| ID: mdl-8456510
ABSTRACT
In erythropoietic protoporphyria, the genetically determined decreased activity of the enzyme ferrochelatase causes accumulation of the photoreactive molecule protoporphyrin in various tissues. Dermatological symptoms are dominant, but in some patients the excess protoporphyrin affects hepato-biliary structures, and a spectrum of changes, which ranges from ultrastructural bile canalicular damage to cirrhosis, can be observed. Most clinical reports have described severe cases with a rapid deterioration and a fatal outcome. We present a case with spontaneous recovery from hepatic decompensation on two occasions with three years interval. The first incidence might have been provoked by hormonal substitution therapy.
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Banco de datos:
MEDLINE
Asunto principal:
Porfiria Hepatoeritropoyética
/
Cirrosis Hepática
Límite:
Female
/
Humans
/
Middle aged
Idioma:
Da
Año:
1993
Tipo del documento:
Article