How is end-stage renal disease treated in pediatric hemolytic uremic syndrome (HUS)?

Updated: Nov 12, 2018
  • Author: Robert S Gillespie, MD, MPH; Chief Editor: Craig B Langman, MD  more...
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Answer

Patients who develop permanent renal failure due to STEC-HUS have a low risk of recurrence and can proceed to renal transplantation similar to patients with most other renal diseases.

Renal transplantation in patients with aHUS is more difficult because of the high risk of recurrence and allograft loss. The risk of recurrence varies with the complement mutation identified; such testing is essential, as is planning and counseling patients about transplantation options. Note the following mutations and recurrence rates (these data were obtained prior to the availability of eculizumab therapy, which may prevent recurrence of aHUS):

  • Factor H mutation: 80-100% recurrence

  • Factor I mutation: 80% recurrence

  • Membrane cofactor protein mutation: 10-20% recurrence

  • No (known) mutation identified: 30% recurrence

Combined liver-kidney transplantation has been reported in patients with high-risk mutations, such as factor H. [32, 33, 34, 35] Liver transplantation alone is an option for patients without renal failure. [32] The principle behind liver transplantation is that the DNA in the donor liver does not have the patient's complement mutation, so it produces normal complement factors.

Prior to the development of eculizumab, kidney transplantation success rates of only 18-33% were reported for patients with high-risk mutations. [36, 37]

Many newer reports describe patients with high-risk mutations who have had successful kidney transplantation, without liver transplants, using eculizumab to prevent recurrence of aHUS. [38, 39, 40]


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