Terminal hepatic failure in erythropoietic protoporphyria.

Abstract:

:Erythropoietic protoporphyria is an inherited disorder characterized biochemically by a deficiency of ferrochelatase, the enzyme that catalyzes the incorporation of ferrous iron into protoporphyrin to form heme. We describe a patient who illustrates the unpredictability of the course of liver disease in erythropoietic protoporphyria. She remained stable for several years after her first evidence of liver function abnormalities. Then, in a period of weeks, hepatic failure developed and she died. Findings of serial liver biopsy specimens showed extensive hepatocellular degeneration and inflammation that appeared in a 10-day period. The factors that cause this rapid deterioration in hepatic function remain unknown. Reported cases of fatal hepatic failure in patients with erythropoietic protoporphyria are reviewed.

journal_name

J Am Acad Dermatol

authors

Mercurio MG,Prince G,Weber FL Jr,Jacobs G,Zaim MT,Bickers DR

doi

10.1016/0190-9622(93)70248-r

subject

Has Abstract

pub_date

1993-11-01 00:00:00

pages

829-33

issue

5 Pt 2

eissn

0190-9622

issn

1097-6787

pii

0190-9622(93)70248-R

journal_volume

29

pub_type

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