Logo image
Back
Renal transplantation in adults with autosomal recessive inheritance of hemolytic uremic syndrome
Journal article   Open access   Peer reviewed

Renal transplantation in adults with autosomal recessive inheritance of hemolytic uremic syndrome

Bernard S. Kaplan, Menelaos Papadimitriou, Joseph H. Brezin, Stephen J. Tomlanovich and Zulkharnain
American journal of kidney diseases, v 30(6), pp 760-765
1997
DOI: https://doi.org/10.1016/S0272-6386(97)90079-2
PMID: 9398118
Featured in Collection :   UN Sustainable Development Goals @ Drexel
url
https://doi.org/10.1016/s0272-6386(97)90079-2View
Published, Version of Record (VoR)CC BY-NC-ND V4.0,  Open

Abstract

recurrence acute renal failure Hemolytic uremic syndrome renal transplantation chrombotic microangiopathy pregnancy autosomal recessive inheritance oral contraceptive
When hemolytic uremic syndrome (HUS) is occasionally inherited in an autosomal recessive mode, this occurs mainly in infants and children. We describe four families in which two adult siblings were affected with HUS in each kindred. HUS first occurred between the ages of 19 to 36 years, and the intervals between the onset of HUS in each sibling pair ranged from 6 months to 6 years. None of the patients had a typical prodrome of bloody diarrhea, and one had a recurrence of HUS before transplantation. All eight patients developed renal failure requiring dialysis and transplantation, and seven patients received kidney transplants. Donor kidneys were from parents, siblings, and cadavers. The initial renal transplants were performed from 6 months to 6 years after the onset of the syndrome. HUS recurred in six of the seven patients 2 weeks to 6.5 years after transplantation regardless of the interval between the onset of HUS and transplantation, the origin of the allograft, or the use of cyclosporin A. The only marker for autosomal recessive HUS is the occurrence of the syndrome in a second sibling several months to many years after its occurrence in the proband. In patient with the autosomal recessive form of HUS, the risk for a recurrence in an allograft is high regardless of the source of the kidney.

Metrics

11 Record Views
27 citations in Web of Science
35 citations in Scopus

Details

UN Sustainable Development Goals (SDGs)

This publication has contributed to the advancement of the following goals:

#3 Good Health and Well-Being

InCites Highlights

Data related to this publication, from InCites Benchmarking & Analytics tool:

Collaboration types
Domestic collaboration
International collaboration
Web of Science research areas
Urology & Nephrology
Logo image