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Titolo:
NEONATAL HEMOCHROMATOSIS - OUTCOMES OF PHARMACOLOGICAL AND SURGICAL THERAPIES
Autore:
SIGURDSSON L; REYES J; KOCOSHIS SA; HANSEN TWR; ROSH J; KNISELY AS;
Indirizzi:
CHILDRENS HOSP PITTSBURGH,DEPT PEDIAT,3705 5TH AVE & DESOTO ST PITTSBURGH PA 15213 CHILDRENS HOSP PITTSBURGH,DEPT SURG PITTSBURGH PA 15213 CHILDRENS HOSP PITTSBURGH,DEPT ANESTHESIOL PITTSBURGH PA 15213 UNIV PITTSBURGH,THOMAS STARZL TRANSPLANT INST PITTSBURGH PA 15260 MT SINAI HOSP,DEPT PEDIAT NEW YORK NY 10029 DENVER AURORA PATHOL ASSOCIATES DENVER CO 00000
Titolo Testata:
Journal of pediatric gastroenterology and nutrition
fascicolo: 1, volume: 26, anno: 1998,
pagine: 85 - 89
SICI:
0277-2116(1998)26:1<85:NH-OOP>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Soggetto:
LIVER-TRANSPLANTATION; FAILURE; DIAGNOSIS;
Keywords:
ANTIOXIDANTS; HEPATIC FAILURE; NEONATAL CHOLESTASIS; NEONATAL HEMOCHROMATOSIS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
15
Recensione:
Indirizzi per estratti:
Citazione:
L. Sigurdsson et al., "NEONATAL HEMOCHROMATOSIS - OUTCOMES OF PHARMACOLOGICAL AND SURGICAL THERAPIES", Journal of pediatric gastroenterology and nutrition, 26(1), 1998, pp. 85-89

Abstract

Background: Neonatal hemochromatosis (NH), also known as perinatal hemochromatosis or neonatal iron storage disease, is a disorder in fetuses and newborn infants. A retrospective study was conducted to report management of patients with NH. Methods: Retrospective analysis was conducted by chart review and by review of histologic material from patients with NH. Results: Neonatal hemochromatosis was diagnosed in 14 patients between 1985 and 1995. All were considered for orthotopic livertransplantation (OLTX). From 1993 onward, all patients were treated with an antioxidant-chelation ''cocktail,'' consisting of deferoxamine,vitamin E, N-acetylcysteine, selenium, and prostaglandin-E1. Of 6 patients with NH diagnosed before 1993, 4 underwent OLTX; only 1 is stillalive. Of 8 patients with NH diagnosed after 1993 and treated with the cocktail, 7 expired before OLTX. One stabilized on therapy, but having never recovered full synthetic liver function, underwent OLTX and is now alive and well. Conclusion: Neonatal hemochromatosis carries a grim prognosis; however, successful OLTX is curative. The use of an antioxidant-chelation cocktail did not improve outcome in the patients studied. Earlier (perinatal) diagnosis may be required for optimal results. Further study of other interventions, including antenatal diagnosis and earlier institution or modification of cocktail therapy appears warranted. (C) 1998 Lippincott-Raven Publishers.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/09/20 alle ore 22:07:18