Abstract

BackgroundThe aim of this study was to investigate the characteristics and outcomes of patients receiving renal replacement therapy for end-stage kidney disease (ESKD) secondary to haemolytic uraemic syndrome (HUS).MethodsThe study included all patients with ESKD who commenced renal replacement therapy in Australia and New Zealand between 15/5/1963 and 31/12/2010, using data from the ANZDATA Registry. HUS ESKD patients were compared with matched controls with an alternative primary renal disease using propensity scores based on age, gender and treatment era.ResultsOf the 58422 patients included in the study, 241 (0.4%) had ESKD secondary to HUS. HUS ESKD was independently associated with younger age, female gender and European race. Compared with matched controls, HUS ESKD was not associated with mortality on renal replacement therapy (adjusted hazard ratio [HR] 1.14, 95% CI 0.87-1.50, p = 0.34) or dialysis (HR 1.34, 95% CI 0.93-1.93, p = 0.12), but did independently predict recovery of renal function (HR 54.01, 95% CI 1.45-11.1, p = 0.008). 130 (54%) HUS patients received 166 renal allografts. Overall renal allograft survival rates were significantly lower for patients with HUS ESKD at 1 year (73% vs 91%), 5 years (62% vs 85%) and 10 years (49% vs 73%). HUS ESKD was an independent predictor of renal allograft failure (HR 2.59, 95% CI 1.70-3.95, p < 0.001). Sixteen (12%) HUS patients experienced failure of 22 renal allografts due to recurrent HUS. HUS ESKD was not independently associated with the risk of death following renal transplantation (HR 0.92, 95% CI 0.35-2.44, p = 0.87).ConclusionsHUS is an uncommon cause of ESKD, which is associated with comparable patient survival on dialysis, an increased probability of renal function recovery, comparable patient survival post-renal transplant and a heightened risk of renal transplant graft failure compared with matched ESKD controls.

Highlights

  • The aim of this study was to investigate the characteristics and outcomes of patients receiving renal replacement therapy for end-stage kidney disease (ESKD) secondary to haemolytic uraemic syndrome (HUS)

  • Using multivariable logistic regression analysis, ESKD secondary to HUS was significantly and independently associated with younger age (p < 0.001), female gender (p < 0.001) and later dialysis era compared with other forms of ESKD (Table 2)

  • A lower probability of HUS ESKD was observed in patients with Asian racial origin (p = 0.03), Aboriginal and Torres Strait Islander (p = 0.001) or Maori and Pacific Islander racial origin (p < 0.001)

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Summary

Introduction

The aim of this study was to investigate the characteristics and outcomes of patients receiving renal replacement therapy for end-stage kidney disease (ESKD) secondary to haemolytic uraemic syndrome (HUS). 10% of HUS cases are not associated with diarrhoea or shiga toxinproducing E. coli and are referred to as atypical HUS [1,5] This heterogeneous disorder may be either familial or sporadic and can be caused or triggered by complement regulatory protein mutations (50%), complement factor H autoantibodies (6-10%), human immunodeficiency virus infection, autoimmune disorders, cardiovascular surgery, transplantation, disseminated malignancy, pregnancy and certain drugs (including calcineurin inhibitors, muromonab-CD3, valacicylovir, clopidogrel, ticlopidine, bleomycin, gemcitabine and cisplatin) [1,2,6,7,8]. There has not been a comprehensive, multi-centre examination of ESKD secondary to HUS

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