A Favorable 3-Year Outcome of Kidney Transplantation in Atypical Hemolytic Uremic Syndrome Associated With a Factor H Mutation: Case Report

Atypical hemolytic-uremic syndrome (aHUS) is an extremely rare complement-mediated disease that belongs to the group of thrombotic microangiopathies (TMA). It often reoccurs after kidney transplantation (KT). Previously, KT was considered contraindicated in both children and adults with aHUS due to high (up to 50% and above) incidence of early graft loss associated with post-transplant recurrent TMA. Introduction of specific complement inhibitor therapy into clinical practice has improved outcomes in patients with aHUS and has significantly reduced the risk of post-transplant recurrence of underlying disease. We describe the clinical observation of a 20-year-old female patient with aHUS associated with antibodies to factor H, a major regulator of complement activation. The patient underwent KT and eculizumab was used for prophylactic purposes. In the postoperative period, the patient developed ureteral necrosis that required reconstructive surgery, followed by graft pyelonephritis. Despite postoperative complications, which were highly likely to trigger uncontrolled complement activation, TMA recurrence was avoided due to early treatment of the complications and prophylactic use of complement inhibitor therapy.

Download Full-text

Favorable long-term outcomes of isolated liver transplantation in a child with atypical hemolytic uremic syndrome caused by a novel complement factor H mutation

Clinical Nephrology ◽

10.5414/cn109138 ◽

2017 ◽

Vol 88 (07) ◽

pp. 52-56 ◽

Cited By ~ 2

Author(s):

Heeyeon Cho ◽

Yeonhee Lee

Keyword(s):

Liver Transplantation ◽

Hemolytic Uremic Syndrome ◽

Atypical Hemolytic Uremic Syndrome ◽

Factor H ◽

Complement Factor ◽

Long Term Outcomes ◽

Uremic Syndrome ◽

Factor H Mutation ◽

Isolated Liver

Download Full-text

Posttransplantation cytomegalovirus-induced recurrence of atypical hemolytic uremic syndrome associated with a factor H mutation: Successful treatment with intensive plasma exchanges and ganciclovir

American Journal of Kidney Diseases ◽

10.1053/j.ajkd.2004.09.012 ◽

2005 ◽

Vol 45 (1) ◽

pp. e12-e15 ◽

Cited By ~ 32

Author(s):

Karolien H. Olie ◽

Timothy H.J. Goodship ◽

René Verlaak ◽

Sandrine Florquin ◽

Jaap W. Groothoff ◽

...

Keyword(s):

Hemolytic Uremic Syndrome ◽

Successful Treatment ◽

Atypical Hemolytic Uremic Syndrome ◽

Factor H ◽

Uremic Syndrome ◽

Factor H Mutation

Download Full-text

Preemptive Use of Eculizumab for Living-Donor Kidney Transplantation in a Child with Atypical Hemolytic Uremic Syndrome

Einstein Journal of Biology and Medicine ◽

10.23861/ejbm201530635 ◽

2016 ◽

Vol 30 (1&2) ◽

pp. 22 ◽

Cited By ~ 1

Author(s):

Oleh Akchurin ◽

Samriti Dogra ◽

Frederick Kaskel ◽

Dominique Jan ◽

Stuart Greenstein ◽

...

Keyword(s):

Kidney Transplantation ◽

Hemolytic Uremic Syndrome ◽

Atypical Hemolytic Uremic Syndrome ◽

Factor H ◽

Complement Factor ◽

Pediatric Kidney Transplantation ◽

Uremic Syndrome ◽

Few Data ◽

Stage Renal Disease ◽

End Stage

Eculizumab is an anti-complement C5 monoclonal antibody that has recently been reported as an effective therapy for atypical hemolytic uremic syndrome. However, few data are available on the preemptive use of this medication in pediatric kidney transplantation. This report describes a successful preemptive use of eculizumab in combination with living unrelated kidney transplanta- tion in a 10-year-old child with end-stage renal disease secondary to atypical hemolytic uremic syndrome who has a complement factor H mutation that has not been previously reported. Further observations and clinical trials are required to address the challenges and areas of uncertainty related to preemptive eculizumab therapy for kidney transplantation in children and adults with atypical hemolytic uremic syndrome.

Download Full-text