Atypical hemolytic uremic syndrome in the Tunisian population

Atypical hemolytic uremic syndrome in the Tunisian population

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 relationsh...

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Published in:International urology and nephrology Vol. 43; no. 2; pp. 559 - 564
Main Authors: Leban, Nadia, Aloui, Sabra, Touati, Dalel, Lakhdhar, Ramzy, Skhiri, Habib, Lefranc, Gerard, Achour, Abdellatif, Elmay, Mezri, Lopez-Trascasa, Margarita, Sanchez-Corral, Pilar, Chibani, Jemni, Haj Khelil, Amel
Format: Journal Article
Language:English
Published: Dordrecht Springer Netherlands 01-06-2011
Springer Nature B.V
Subjects:
Adult
Complement System Proteins - analysis
Female
Hemolytic-Uremic Syndrome - blood
Hemolytic-Uremic Syndrome - diagnosis
Humans
Male
Medicine
Medicine & Public Health
Middle Aged
Nephrology
Nephrology – Case Report
Retrospective Studies
Tunisia
Urology
Tunisia
Atypical hemolytic uremic syndrome
Tunisian population
Complement system
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Summary: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.
Bibliography:ObjectType-Case Study-2
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ISSN:0301-1623
1573-2584
DOI:10.1007/s11255-010-9754-3