Epidemiology, clinical presentation, and pathophysiology of atypical and recurrent hemolytic uremic syndrome

Lothar Bernd Zimmerhackl, Nesir Besbas, Therese Jungraithmayr, Nicole Van De Kar, Helge Karch, Diana Karpman, Daniel Landau, Chantal Loirat, Willem Proesmans, Friederike Prüfer, Gianfranco Rizzoni, Mark C. Taylor

Research output: Contribution to journalArticlepeer-review

85 Scopus citations


Hemolytic uremic syndrome (HUS) includes a heterogeneous group of hemolytic disorders. Among the identified causes of HUS are infections, particularly infections with Shiga toxin-producing Escherichia coli (STEC), complement disorders, and disorders interfering with the degradation of von Willebrand factor (VWF). Other causes for atypical HUS include the cobalamin metabolism; pregnancy/hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP); drugs; and other disorders (e.g., systemic diseases appearing as HUS, such as systemic lupus erythematosus and rejection after transplantation). The group not related to STEC is often also called atypical HUS. Most of the occurrences of infectious HUS have only one episode. Recurrent episodes (recurrent HUS) have strong relationships to diseases of the complement system. In these two subgroups the prognosis is poor, with severe renal insufficiency, together with the need for renal replacement therapy. Severe arterial hypertension is common. Treatment options are limited. To better define this group of patients, the European Society for Pediatric Nephrology supported an initiative to develop a European HUS registry. In this registry, 167 patients were acquired; 73 were female (43.8%). The year of onset of the disease ranged from 1974 to 2005. The prevalence of atypical HUS/recurrent HUS can be calculated as 3.3 per million child population (< 18 years). Underlying disorders included factor H, factor I, MCP-1, pneumococci, and von Willebrand factor disturbances. In 33 patients at least one renal transplantation was performed (total, 55 kidneys); 18% were successful and 73% demonstrated recurrence or thrombosis. Treatment options were plasma substitution or plasmapheresis. Despite continued efforts, transplantation is not recommended at present for these patients. Living-related transplantation should be abandoned. New therapeutic strategies are urgently needed.

Original languageEnglish
Pages (from-to)113-120
Number of pages8
JournalSeminars in Thrombosis and Hemostasis
Issue number2
StatePublished - 1 Jan 2006
Externally publishedYes


  • Complement system
  • Enterohemorrhagic Escherichia coli
  • Hemolytic uremic syndrome
  • Plasma treatment
  • Recurrence
  • Transplantation
  • Von Willebrand factor

ASJC Scopus subject areas

  • Hematology
  • Cardiology and Cardiovascular Medicine


Dive into the research topics of 'Epidemiology, clinical presentation, and pathophysiology of atypical and recurrent hemolytic uremic syndrome'. Together they form a unique fingerprint.

Cite this