Successful treatment of severe hepatic impairment in erythropoietic protoporphyria: A case report and review of literature.
World J Hepatol
; 16(6): 966-972, 2024 Jun 27.
Article
in En
| MEDLINE
| ID: mdl-38948434
ABSTRACT
BACKGROUND:
Erythropoietic protoporphyria (EPP) is a rare genetic disorder stemming from ferrochelatase gene mutations, which leads to abnormal accumulation of protoporphyrin IX primarily in erythrocytes, skin, bone marrow and liver. Although porphyria-related severe liver damage is rare, its consequences can be severe with limited treatment options. CASESUMMARY:
This case study highlights a successful intervention for a 35-year-old male with EPP-related liver impairment, employing a combination of red blood cell (RBC) exchange and therapeutic plasma exchange (TPE). The patient experienced significant symptom relief and a decrease in bilirubin levels following multiple PE sessions and an RBC exchange.CONCLUSION:
The findings suggest that this combined approach holds promise for managing severe hepatic impairment in EPP.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Language:
En
Journal:
World J Hepatol
Year:
2024
Document type:
Article
Affiliation country: