Haemolytic uraemic syndrome

Definition

Haemolytic uraemic syndrome (HUS) is a syndrome associated with microvascular diseases of the kidney and is characterized by haemolytic uraemia, thrombocytopenia and acute renal failure. Most cases of HUS are caused by bacterial or viral infection, although more-severe cases (atypical HUS) can result from genetic defects in complement activation.

Latest Research and Reviews

  • Reviews |

    Autoantibodies against complement factor H (FH), the main plasma regulatory protein of the alternative pathway of the complement system, are associated with atypical haemolytic uraemic syndrome and C3 glomerulopathy. Here, Arvind Bagga and colleagues describe the prevalence, mechanisms, features, therapies and outcomes of kidney diseases mediated by anti-FH antibodies, and propose an approach to evaluate and manage these diseases.

    • Marie-Agnes Dragon Durey
    • , Aditi Sinha
    • , Shambhuprasad Kotresh Togarsimalemath
    •  & Arvind Bagga
  • Reviews |

    Renal transplantation is the optimal form of renal replacement therapy for children with end-stage renal disease; however, disease recurrence can lead to graft loss, morbidity and death. In this Review, Justine Bacchetta and Pierre Cochat provide an update on the epidemiology, pathophysiology, effects and management of disease recurrence after paediatric renal transplantation. They also describe pretransplantation and post-transplantation risk-reduction strategies that aim to minimize the possibility of disease recurrence, and thus improve both graft and patient outcomes.

    • Justine Bacchetta
    •  & Pierre Cochat
  • Reviews |

    Here, Zuber et al., on behalf of the French Study Group for aHUS/C3G, discuss the role of eculizumab in the treatment of atypical haemolytic uraemic syndrome (aHUS). They review data from case reports and preliminary data from prospective trials, present their opinions, and describe issues that require further study. In addition, they discuss the potential use of eculizumab in C3 glomerulopathies.

    • Julien Zuber
    • , Fadi Fakhouri
    • , Lubka T. Roumenina
    • , Chantal Loirat
    •  & Véronique Frémeaux-Bacchi
  • Research |

    Richard Lifton, Véronique Frémeaux-Bacchi and colleagues report that recessive mutations in DGKE cause atypical hemolytic-uremic syndrome with an early age of onset progressing to chronic kidney disease. The authors propose that loss of DGKE results in a prothrombotic state that underlies disease pathogenesis.

    • Mathieu Lemaire
    • , Véronique Frémeaux-Bacchi
    • , Franz Schaefer
    • , Murim Choi
    • , Wai Ho Tang
    • , Moglie Le Quintrec
    • , Fadi Fakhouri
    • , Sophie Taque
    • , François Nobili
    • , Frank Martinez
    • , Weizhen Ji
    • , John D Overton
    • , Shrikant M Mane
    • , Gudrun Nürnberg
    • , Janine Altmüller
    • , Holger Thiele
    • , Denis Morin
    • , Georges Deschenes
    • , Véronique Baudouin
    • , Brigitte Llanas
    • , Laure Collard
    • , Mohammed A Majid
    • , Eva Simkova
    • , Peter Nürnberg
    • , Nathalie Rioux-Leclerc
    • , Gilbert W Moeckel
    • , Marie Claire Gubler
    • , John Hwa
    • , Chantal Loirat
    •  & Richard P Lifton
    Nature Genetics 45, 531–536
  • Reviews |

    Shiga toxin-producing Escherichia coli-associated haemolytic uraemic syndrome (STEC-HUS) is associated with renal and neurological injury. This Review summarizes the pathophysiology and clinical presentation of STEC-HUS and its acute and long-term effects on the kidney and central nervous system. The authors also describe the experience of a single centre that was affected by the STEC-HUS outbreak in Germany in 2011.

    • Howard Trachtman
    • , Catherine Austin
    • , Maria Lewinski
    •  & Rolf A. K. Stahl
  • Reviews |

    Postrenal transplant hemolytic uremic syndrome (HUS) can occur as a recurrent or de novo disease. Improved understanding of the mechanisms by which atypical HUS occurs after transplantation should result in the improved management of affected patients. In this Review, Zuber and colleagues discuss recent advances in our understanding of the pathogenesis of atypical HUS and describe diagnostic and therapeutic considerations with regard to renal transplantation in these patients.

    • Julien Zuber
    • , Moglie Le Quintrec
    • , Rebecca Sberro-Soussan
    • , Chantal Loirat
    • , Véronique Frémeaux-Bacchi
    •  & Christophe Legendre

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