Review article
ANDERSON-FABRIJEVA BOLEST I TRANSPLANTACIJA BUBREGA
NIKOLINA BAŠIĆ-JUKIĆ
; Zagreb University Hospital Center and School of Medicine, University of Zagreb, Department of Nephrology, Arterial Hypertension and Dialysis Zagreb, Croatia
PETAR KES
; Zagreb University Hospital Center and School of Medicine, University of Zagreb, Department of Nephrology, Arterial Hypertension and Dialysis Zagreb, Croatia
TVRTKO HUDOLIN
; Zagreb University Hospital Center, Department of Urology, Zagreb, Croatia
INES MESAR
; Zagreb University Hospital Center and School of Medicine, University of Zagreb, Department of Nephrology, Arterial Hypertension and Dialysis Zagreb, Croatia
MARIJANA ĆORIĆ
; Zagreb University Hospital Center, Department of Pathology, Zagreb, Croatia
ŽELJKO KAŠTELAN
; Zagreb University Hospital Center, Department of Urology, Zagreb, Croatia
Abstract
Anderson-Fabrijeva bolest (AFB) je rijetka bolest nakupljanja koja se prenosi putem X kromosoma, a dovodi do progresivnog nakupljanja globotriaozilceramida u visceralnim organima i endotelu krvnih žila. Bolest prevenstveno zahvaća muškarce. Dugo se smatralo da su žene asimptomatski nositelji, ali novija istraživanja pokazuju da se manifestna bolest javlja i u značajnom broju žena. Prikazujemo posttransplantacijski tijek u bolesnica koje su dobile presadak od darivatelja s Anderson-Fabrijevom bolesti, te u primatelja presatka kojemu su vlastiti bubrezi zatajili zbog Anderson-Fabrijeve bolesti.
Keywords
Anderson-Fabrijeva bolest; transplantacija bubrega; ishod
Hrčak ID:
89625
URI
Publication date:
1.3.2011.
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