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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


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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

https://hrcak.srce.hr/89625

Publication date:

1.3.2011.

Article data in other languages: english

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