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Titolo:
TERMINAL HEPATIC-FAILURE IN ERYTHROPOIETIC PROTOPORPHYRIA
Autore:
MERCURIO MG; PRINCE G; WEBER FL; JACOBS G; ZAIM MT; BICKERS DR;
Indirizzi:
UNIV HOSP CLEVELAND,DEPT DERMATOL,2074 ABINGTON RD CLEVELAND OH 44106 CASE WESTERN RESERVE UNIV HOSP,DEPT DERMATOL CLEVELAND OH 44106 CASE WESTERN RESERVE UNIV HOSP,DEPT PATHOL CLEVELAND OH 44106 CASE WESTERN RESERVE UNIV HOSP,DEPT MED,DIV GASTROENTEROL CLEVELAND OH 44106
Titolo Testata:
Journal of the American Academy of Dermatology
fascicolo: 5, volume: 29, anno: 1993,
parte:, 2
pagine: 829 - 833
SICI:
0190-9622(1993)29:5<829:THIEP>2.0.ZU;2-J
Fonte:
ISI
Lingua:
ENG
Soggetto:
LIVER-TRANSPLANTATION; IRON THERAPY; CIRRHOSIS; DISEASE; HEPATOPATHY; PORPHYRIAS; PATIENT; DEATH;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
40
Recensione:
Indirizzi per estratti:
Citazione:
M.G. Mercurio et al., "TERMINAL HEPATIC-FAILURE IN ERYTHROPOIETIC PROTOPORPHYRIA", Journal of the American Academy of Dermatology, 29(5), 1993, pp. 829-833

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 andshe died. Findings of serial liver biopsy specimens showed extensive hepatocellular degeneration and inflammation that appeared in a 10-dayperiod. 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.

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Documento generato il 03/12/20 alle ore 15:17:18