title: Atypical hemolytic uremic syndrome in the Tunisian population.
abstract: 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.
authors.affiliation: Biochemistry and Molecular Biology Laboratory, Faculty of Pharmacy, AV. Avicenne, 5019 Monastir, Tunisia. lebannadia@yahoo.fr
authors.forename: Nadia
authors.initials: N
authors.lastname: Leban
authors.forename: Sabra
authors.initials: S
authors.lastname: Aloui
authors.forename: Dalel
authors.initials: D
authors.lastname: Touati
authors.forename: Ramzy
authors.initials: R
authors.lastname: Lakhdhar
authors.forename: Habib
authors.initials: H
authors.lastname: Skhiri
authors.forename: Gerard
authors.initials: G
authors.lastname: Lefranc
authors.forename: Abdellatif
authors.initials: A
authors.lastname: Achour
authors.forename: Mezri
authors.initials: M
authors.lastname: Elmay
authors.forename: Margarita
authors.initials: M
authors.lastname: Lopez-Trascasa
authors.forename: Pilar
authors.initials: P
authors.lastname: Sanchez-Corral
authors.forename: Jemni
authors.initials: J
authors.lastname: Chibani
authors.forename: Amel
authors.initials: A
authors.lastname: Haj Khelil
mesh_terms: 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
chemical_list: D003165:Complement System Proteins