title: Diagnosis and classification of hemolytic uremic syndrome: the Hungarian experience.
abstract: BACKGROUND
  Hemolytic uremic syndrome (HUS) is a rare disease with various etiologies, making the identification of the specific forms and appropriate treatment difficult. Therefore, clinical and laboratory data from these patients need to be analyzed in national and international registries. Herein we have described 47 Hungarian HUS patients with detailed laboratory and clinical data obtained between 2008 and 2010.
  
  
  METHODS
  Blood samples and clinical data of 47 patients with HUS diagnosed according to characteristic clinical signs were submitted for diagnostic evaluation, including complement protein and genetic analysis, measurement of ADAMTS13 activity and antibody analysis against O157LPS and factor H.
  
  
  RESULTS
  There were 8 patients with typical diarrhea-positive HUS; 13 with atypical HUS (aHUS) and 26 with secondary HUS/thrombotic thrombocytopenic purpura group characterized by signs of complement consumption and decreased ADAMTS13 activity. Thus, decreased total alternative pathway activity is a promising diagnostic parameter with good sensitivity for aHUS.
  
  
  CONCLUSIONS
  These observations highlight the requirement for multiple diagnostic tests together with clinical data to identify the specific cause of HUS. Because the long-term prognosis of aHUS, eg, graft survival after renal transplantation, may vary according to the molecular etiology, it is important for all affected patients to undergo a detailed molecular diagnosis of the disease. There is a clear clinical need for the development and application of novel assay in this field to allow more rapid efficient diagnosis of patients who undergo a first episode of HUS.
authors.affiliation: First Department of Pediatrics, Semmelweis University, Budapest, Hungary.
authors.forename: G S
authors.initials: GS
authors.lastname: Reusz
authors.forename: A J
authors.initials: AJ
authors.lastname: Szabó
authors.forename: M
authors.initials: M
authors.lastname: Réti
authors.forename: Z
authors.initials: Z
authors.lastname: Györke
authors.forename: Á
authors.initials: Á
authors.lastname: Szilágyi
authors.forename: P
authors.initials: P
authors.lastname: Farkas
authors.forename: Z
authors.initials: Z
authors.lastname: Prohászka
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000907:Antibodies, Bacterial / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001798:Blood Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003176:Complement C3 / Q000032:analysis; D003180:Complement C3b Inactivator Proteins / Q000235:genetics; D011415:Complement Factor B / Q000032:analysis; D017242:Complement Factor H / Q000032:analysis / Q000276:immunology; D017244:Complement Factor I / Q000032:analysis; D019453:Escherichia coli O157 / Q000276:immunology; D005260:Female; D020022:Genetic Predisposition to Disease; D006463:Hemolytic-Uremic Syndrome / Q000097:blood / Q000145:classification* / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics; D006801:Humans; D006814:Hungary / Q000453:epidemiology; D007223:Infant; D008070:Lipopolysaccharides / Q000276:immunology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D055815:Young Adult
chemical_list: D000907:Antibodies, Bacterial; D001323:Autoantibodies; D015415:Biomarkers; D001798:Blood Proteins; C493495:CFH protein, human; C517621:CFHR1 protein, human; C105970:CFHR3 protein, human; D003176:Complement C3; D003180:Complement C3b Inactivator Proteins; D008070:Lipopolysaccharides; D017242:Complement Factor H; C493611:CFI protein, human; D017244:Complement Factor I; D011415:Complement Factor B; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human