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Clinical Practice Guidelines for the management of atypical Haemolytic Uraemic Syndrome in the United Kingdom
Author(s) -
Taylor C. Mark,
Machin Sam,
Wigmore Stephen J.,
Goodship Tim H. J.
Publication year - 2010
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2009.07916.x
Subject(s) - atypical hemolytic uremic syndrome , medicine , transplantation , intensive care medicine , complement system , disease , autoantibody , immunology , presentation (obstetrics) , pediatrics , antibody , surgery
Summary Atypical haemolytic uraemic syndrome (aHUS) is associated with a poor prognosis with regard to survival at presentation, recovery of renal function and transplantation. It is now established that aHUS is a disease of complement dysregulation with mutations in the genes encoding both complement regulators and activators, and autoantibodies against the complement regulator factor H. Identification of the underlying molecular abnormality in an individual patient can now help to guide their future management. In these guidelines we make recommendations for the investigation and management of aHUS patients both at presentation and in the long‐term. We particularly address the role of renal transplantation alone and combined liver‐kidney transplantation.