Atypical hemolytic uremic syndrome in the Tunisian population.
BACKGROUND
Hemolytic uremic syndrome consists of a triad of acquired hemolytic anemia, thrombocytopenia and renal failure.


OBJECTIVE
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.


CONCLUSIONS
Nephrologists should be aware of autoimmune conditions and genetic abnormalities of the complement regulatory genes as possible pathogenic mechanisms in atypical HUS patients.
Leban
Nadia
N
Biochemistry and Molecular Biology Laboratory, Faculty of Pharmacy, AV. Avicenne, 5019 Monastir, Tunisia. lebannadia@yahoo.fr
Aloui
Sabra
S
Touati
Dalel
D
Lakhdhar
Ramzy
R
Skhiri
Habib
H
Lefranc
Gerard
G
Achour
Abdellatif
A
Elmay
Mezri
M
Lopez-Trascasa
Margarita
M
Sanchez-Corral
Pilar
P
Chibani
Jemni
J
Haj Khelil
Amel
A
D000328:Adult; D003165:Complement System Proteins / Q000032:analysis; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000097:blood / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014416:Tunisia
D003165:Complement System Proteins