title: The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome Registry: the Swiss connection.
abstract: OBJECTIVE
  Thrombotic thrombocytopenic purpura (TTP) was initially described as an uncommon and usually fatal disorder. With effective treatment it is more frequently diagnosed, the clinical presentations are more diverse, and long-term sequelae are becoming recognized.
  
  
  METHODS
  Patient data are from The Oklahoma TTP-hemolytic uremic syndrome (HUS) Registry, an inception cohort of 348 consecutive patients with their first episode of clinically diagnosed TTP or HUS, 1989-2006. The Registry enrolls all patients in a defined region who are diagnosed with TTP or HUS and for whom plasma exchange treatment is requested. ADAMTS13 activity has been analyzed on 235 (93%) of 254 patients since 1995 at the University of Berne, Switzerland. Patients are described by clinical categories, related to their associated conditions and clinically apparent etiologies, and by the presence of severe ADAMTS13 deficiency.
  
  
  CONCLUSIONS
  The clinical spectrum of syndromes described as TTP is variable with multiple etiologies. Advances in clinical and laboratory investigation have provided better understanding of the pathogenesis of these syndromes, their clinical evaluation and management, and their long-term outcomes. In addition to new information about TTP, these studies provide a model for translational research to define the complete community spectrum of uncommon disorders.
authors.affiliation: Colleges of Public Health, The University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA. james-george@ouhsc.edu
authors.forename: James N
authors.initials: JN
authors.lastname: George
authors.forename: Johanna A
authors.initials: JA
authors.lastname: Kremer Hovinga
authors.forename: Deirdra R
authors.initials: DR
authors.lastname: Terrell
authors.forename: Sara K
authors.initials: SK
authors.lastname: Vesely
authors.forename: Bernhard
authors.initials: B
authors.lastname: Lämmle
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency / Q000235:genetics / Q000378:metabolism; D006463:Hemolytic-Uremic Syndrome / Q000145:classification / Q000150:complications* / Q000175:diagnosis* / Q000378:metabolism; D006801:Humans; D009825:Oklahoma; D011697:Purpura, Thrombotic Thrombocytopenic / Q000145:classification / Q000150:complications* / Q000175:diagnosis* / Q000378:metabolism; D012042:Registries; D013557:Switzerland
chemical_list: D051722:ADAM Proteins