Medizinische Universität Innsbruck
HUS - Hemolytic Uremic Syndrome
HUS - Hemolytic Uremic Syndrome
HUS - Hämolytisch Urämisches Syndrom HUS - Hämolytisch Urämisches Syndrom
.:: RESEARCH ::.

Typical hemolytic uremic syndrome (HUS):
In 1997 a registry was established within the HUS-Net Study for data of patients with typical, i.e. diarrhea-associated, HUS.

Aims of the study are:
  • Epidemiology of D+HUS cases including age at disease onset, pathogen and serotypes of Stx+ D+ HUS cases
  • Cinical manifestation of the disease
  • Treatment of the classical HUS
  • Follow-up for the discovery of long term sequelae
  • Risk factors for long term sequelae
  • Establishment of a comprehensive evidence based model for aHUS pathogenesis

Atypical hemolytic uremic syndrome (aHUS):
The HUS-Net Study also compiles data concerning the genetics and the involvement of complement in this severe kidney disease.

Aims of this study are:
  • Genetic analysis of patients with aHUS
  • Establishment of a comprehensive evidence based mode for aHUS pathogenesis
  • Clinical course and outcome of the recurrent aHUS
  • Identification and validation of genetic and acquired risk factors for aHUS
  • Differentiation of the different subgroups within this disease
  • Clinical course and outcome of the recurrent aHUS
  • Establishment of a "indication profile" for an individualized aHUS therapy
  • Improvement of therapy in general
  • Prevention of permanent endstage renal disease in aHUS patients
  • Evaluation of a renal transplantation without recurrence for aHUS patients on dialysis

Typical hemolytic uremic syndrome

Zimmerhackl LB, Rosales A, Hofer J, Riedl M, Jungraithmayr T, Mellmann A, Bielaszewska M, Karch H. Enterohemorrhagic Escherichia coli O26:H11-Associated Hemolytic Uremic Syndrome: Bacteriology and Clinical Presentation. Semin Thromb Hemost. 2010 Sep;36(6):586-93. Epub 2010 Sep 23.

Orth D, Khan AB, Naim A, Grif K, Brockmeyer J, Karch H, Joannidis M, Clark SJ, Day AJ, Fidanzi S, Stoiber H, Dierich MP, Zimmerhackl LB, Würzner R. Shiga toxin activates complement and binds factor H: evidence for an active role of complement in hemolytic uremic syndrome. J Immunol. 2009 May 15;182(10):6394-400.

Orth D, Grif K, Zimmerhackl LB, Würzner R. Sorbitol-fermenting Shiga toxin-producing Escherichia coli O157 in Austria. Wien Klin Wochenschr. 2009;121(3-4):108-12.

Khan AB, Naim A, Orth D, Grif K, Mohsin M, Prager R, Dierich MP, Würzner R. Serine protease espP subtype alpha, but not beta or gamma, of Shiga toxin-producing Escherichia coli is associated with highly pathogenic serogroups. Int J Med Microbiol. 2009 Apr;299(4):247-54.

Orth D, Grif K, Zimmerhackl LB, Würzner R. Prevention and treatment of enterohemorrhagic Escherichia coli infections in humans. Expert Rev Anti Infect Ther. 2008 Feb;6(1):101-8.

Scheiring J, Andreoli SP, Zimmerhackl LB. Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS). Pediatr Nephrol. 2008 Oct;23(10):1749-60.

Orth D, Grif K, Khan AB, Naim A, Dierich MP, Würzner R. The Shiga toxin genotype rather than the amount of Shiga toxin or the cytotoxicity of Shiga toxin in vitro correlates with the appearance of the hemolytic uremic syndrome. Diagn Microbiol Infect Dis. 2007 Nov;59(3):235-42.

Orth D, Würzner R. What makes an enterohemorrhagic Escherichia coli? Clin Infect Dis. 2006 Nov 1;43(9):1168-9.

Orth D, Grif K, Dierich MP, Würzner R. Sorbitol-fermenting Shiga toxin-producing Escherichia coli O157: indications for an animal reservoir. Epidemiol Infect. 2006 Aug;134(4):719-23.

Grif K, Orth D, Lederer I, Berghold C, Roedl S, Mache CJ, Dierich MP, Würzner R. Importance of environmental transmission in cases of EHEC O157 causing hemolytic uremic syndrome. Eur J Clin Microbiol Infect Dis. 2005 Apr;24(4):268-71.

Gerber A, Karch H, Allerberger F, Verweyen HM, Zimmerhackl LB. Clinical course and the role of shiga toxin-producing Escherichia coli infection in the hemolytic-uremic syndrome in pediatric patients, 1997-2000, in Germany and Austria: a prospective study.J Infect Dis. 2002 Aug 15;186(4):493-500.

Complement in typical hemolytic uremic syndrome

Orth D, Würzner R. Complement in typical hemolytic uremic syndrome. Semin Thromb Hemost. 2010 Sep;36(6):620-4.

Orth D, Ehrlenbach S, Brockmeyer J, Khan AB, Huber G, Karch H, Sarg B, Lindner H, Würzner R. EspP, a serine protease of enterohemorrhagic Escherichia coli, impairs complement activation by cleaving complement factors C3/C3b and C5. Infect Immun. 2010 Oct;78(10):4294-301.

Atypical hemolytic uremic syndrome

Rosales A, Riedl M, Zimmerhackl LB. Thrombotic microangiopathy: atypical HUS: current diagnostic and therapeutic approaches. Nat Rev Nephrol. 2010 Sep;6(9):504-6.
Zimmerhackl LB, Hofer J, Cortina G, Mark W, Würzner R, Jungraithmayr TC, Khursigara G, Kliche KO, Radauer W. Prophylactic eculizumab after renal transplantation in atypical hemolytic-uremic syndrome. N Engl J Med. 2010 May 6;362(18):1746-8

Rosales A, Hofer J, Zimmerhackl LB: Complement regulation disorders and outcome after transplantation in children with atypical hemolytic uremic syndrome. Salud I Ciencia 2010;17:320-324.

Scheiring J, Rosales A, Zimmerhackl LB. Clinical practice. Today's understanding of the haemolytic uraemic syndrome. Eur J Pediatr. 2010 Jan;169(1):7-13. Epub 2009 Aug 26.

Lhotta K, Janecke AR, Scheiring J, Petzlberger B, Giner T, Fally V, Würzner R, Zimmerhackl LB, Mayer G, Fremeaux-Bacchi V. A large family with a gain-of-function mutation of complement C3 predisposing to atypical hemolytic uremic syndrome, microhematuria, hypertension and chronic renal failure. Clin J Am Soc Nephrol. 2009 Aug;4(8):1356-62.

Nürnberger J, Philipp T, Witzke O, Opazo Saez A, Vester U, Baba HA, Kribben A, Zimmerhackl LB, Janecke AR, Nagel M, Kirschfink M. Eculizumab for atypical hemolytic-uremic syndrome. N Engl J Med. 2009 Jan 29;360(5):542-4.

Ariceta G, Besbas N, Johnson S, Karpman D, Landau D, Licht C, Loirat C, Pecoraro C, Taylor CM, Van de Kar N, Vandewalle J, Zimmerhackl LB; European Paediatric Study Group for HUS. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol. 2009 Apr;24(4):687-96.

Zimmerhackl LB, Scheiring J, Prüfer F, Taylor CM, Loirat C. Renal transplantation in HUS patients with disorders of complement regulation. Pediatr Nephrol. 2007 Jan;22(1):10-6.

Besbas N, Karpman D, Landau D, Loirat C, Proesmans W, Remuzzi G, Rizzoni G, Taylor CM, Van de Kar N, Zimmerhackl LB; European Paediatric Research Group for HUS. A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders. Kidney Int. 2006 Aug;70(3):423-31.

Prüfer F, Scheiring J, Sautter S, Jensen DB, Treichl R, Würzner R, Zimmerhackl LB. Terminal complement complex (C5b-9) in children with recurrent hemolytic uremic syndrome. Semin Thromb Hemost. 2006 Mar;32(2):121-7.

Zimmerhackl LB, Besbas N, Jungraithmayr T, van de Kar N, Karch H, Karpman D, Landau D, Loirat C, Proesmans W, Prüfer F, Rizzoni G, Taylor MC; European Study Group for Haemolytic Uraemic Syndromes and Related Disorders. Epidemiology, clinical presentation, and pathophysiology of atypical and recurrent hemolytic uremic syndrome. Semin Thromb Hemost. 2006 Mar;32(2):113-20.

FH antibodies

Dragon-Durey MA, Blanc C, Garnier A, Hofer J, Sethi SK, Zimmerhackl LB. Anti-factor H autoantibody-associated hemolytic uremic syndrome: review of literature of the autoimmune form of HUS. Semin Thromb Hemost. 2010 Sep;36(6):633-40.

Skerka C, Zipfel PF, Müller D, Micklisch S, Riedl M, Zimmerhackl LB, Hofer J. The autoimmune disease DEAP-hemolytic uremic syndrome. Semin Thromb Hemost. 2010 Sep;36(6):625-32. Epub 2010 Sep 23.

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