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Heterotopic Auxiliary Rat Liver Transplantation With Flow-regulated Portal Vein Arterialization in Acute Hepatic Failure
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Favorable long-term outcome after liver-kidney transplant for recurrent hemolytic uremic syndrome associated with a

J M Saland1, S H Emre, B L Shneider

  • 1Department of Pediatrics, The Mount Sinai Medical Center, New York, USA. jeff.saland@mssm.edu

American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons
|August 8, 2006
PubMed

Insights

A child with atypical hemolytic uremic syndrome (HUS) due to complement factor H mutations underwent a combined liver-kidney transplant. This successful procedure offered a favorable long-term outcome, overcoming previous transplant failures.

Area of Science:

  • Nephrology
  • Transplantation Immunology
  • Genetics

Background:

  • Atypical hemolytic uremic syndrome (HUS) is a severe thrombotic microangiopathy often linked to genetic complement system dysregulation.
  • Complement factor H (CFH) mutations are a significant cause of HUS, leading to renal failure and transplant complications.

Observation:

  • A pediatric patient with CFH mutations experienced rapid progression to end-stage renal disease (ESRD) and graft loss after a kidney transplant due to recurrent HUS.
  • Dialysis posed significant risks including access failure and bacteremia.

Findings:

  • Genetic analysis revealed compound heterozygous mutations in the complement factor H gene (c.2918G > A, Cys973Tyr and c.3590T > C, Val1197Ala).
  • A combined liver-kidney transplant with pre-operative plasma exchange resulted in excellent graft function maintained for over two years.

Implications:

  • Combined liver-kidney transplantation is a viable therapeutic option for HUS patients with CFH mutations who have experienced graft failure.
  • This approach offers a promising long-term solution for managing this complex condition and improving patient survival.

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