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- Title
Use of eculizumab and plasma exchange in successful combined liver-kidney transplantation in a case of atypical HUS associated with complement factor H mutation.
- Authors
Tran, Ha; Chaudhuri, Abanti; Concepcion, Waldo; Grimm, Paul
- Abstract
Background: Atypical hemolytic uremic syndrome (aHUS) evolves into end-stage renal failure in nearly half of affected patients and is associated with defective regulation of the alternative complement pathway. Patients with a complement factor H (CFH) mutation have a 30-100% risk of graft loss due to aHUS recurrence or graft thrombosis. Since CFH is produced predominantly by the liver, combined liver-kidney transplant is a curative treatment option. One major unexpected risk includes liver failure secondary to uncontrolled complement activation. We report a successful combined liver-kidney transplantation with perioperative plasma exchange and use of the humanized anti-C5 monoclonal antibody eculizumab. Case Diagnosis/Treatment: An 11-month-old female presented with oliguric renal failure after 3 weeks of flu-like symptoms in the absence of diarrhea. Following the identification of Escherichia coli 0157:H7 in her stool, she was discharged home on peritoneal dialysis with a diagnosis of Shiga toxin-associated HUS. Three months later, she developed severe anemia, thrombocytopenia, and neurological involvement. aHUS was diagnosed and confirmed, and genetic testing revealed a mutation in CFH SCR20. Once donor organs became available, she received preoperative plasma exchange followed by eculizumab infusion with intra-operative fresh frozen plasma prior to combined liver-kidney transplant. At 19 months post-transplant, she continues to have excellent allograft and liver function without signs of disease recurrence. Conclusion: Perioperative use of eculizumab in conjunction with plasma exchange during simultaneous liver-kidney transplant can be used to inhibit terminal complement activity, thereby optimizing successful transplantation by reducing the risk of graft thrombosis.
- Subjects
HEMOLYTIC-uremic syndrome treatment; HEMOLYTIC-uremic syndrome diagnosis; COMPLEMENT (Immunology); COUGH; FEVER; HEMOLYTIC-uremic syndrome; KIDNEY transplantation; LIVER transplantation; MONOCLONAL antibodies; GENETIC mutation; PLASMA exchange (Therapeutics); GENETICS
- Publication
Pediatric Nephrology, 2014, Vol 29, Issue 3, p477
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-013-2630-5