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International Urology and Nephrology
Published in: International Urology and Nephrology 2/2011

01-06-2011 | Nephrology – Case Report

Atypical hemolytic uremic syndrome in the Tunisian population

Authors: Nadia Leban, Sabra Aloui, Dalel Touati, Ramzy Lakhdhar, Habib Skhiri, Gerard Lefranc, Abdellatif Achour, Mezri Elmay, Margarita Lopez-Trascasa, Pilar Sanchez-Corral, Jemni Chibani, Amel Haj Khelil

Published in: International Urology and Nephrology | Issue 2/2011

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Abstract

Background

Hemolytic uremic syndrome consists of a triad of acquired hemolytic anemia, thrombocytopenia and renal failure.

Aim

Our objectives were to determine epidemiology, clinical and laboratory characteristics of patients with atypical hemolytic uremic syndrome (aHUS) to determine the relationship between the complement protein deficit and aHUS in the Tunisian population.

Methods

We studied retrospectively four cases of atypical HUS in adults admitted in the Nephrology Department of Fattouma Bourguiba Universitary Hospital in Monastir between 2000 and 2008.

Results

Three patients had renal failure that required dialysis. One of them received kidney transplantation with no further recurrence of aHUS. Three patients had normal C3, C4, CFH, and FB levels, and in all patients anti-FH autoantibodies were absent. The kidney biopsy of one patient showed in addition to lupus glomerulonephritis histological findings consistent with TMA. A decrease in C3, C4 and CFH levels in this patient was found both before and after the cure.

Conclusion

Nephrologists should be aware of autoimmune conditions and genetic abnormalities of the complement regulatory genes as possible pathogenic mechanisms in atypical HUS patients.
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Metadata
Title
Atypical hemolytic uremic syndrome in the Tunisian population
Authors
Nadia Leban
Sabra Aloui
Dalel Touati
Ramzy Lakhdhar
Habib Skhiri
Gerard Lefranc
Abdellatif Achour
Mezri Elmay
Margarita Lopez-Trascasa
Pilar Sanchez-Corral
Jemni Chibani
Amel Haj Khelil
Publication date
01-06-2011
Publisher
Springer Netherlands
Published in
International Urology and Nephrology / Issue 2/2011
Print ISSN: 0301-1623
Electronic ISSN: 1573-2584
DOI
https://doi.org/10.1007/s11255-010-9754-3

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