title: Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome.
abstract: OBJECTIVE
  A male infant with a family history of thrombotic microangiopathy developed atypical hemolytic uremic syndrome (aHUS).
  
  
  METHODS
  Case report.
  
  
  RESULTS
  Genetic analysis demonstrated a heterozygous mutation (S1191L) of CFH, the gene coding complement factor H (CFH). The child suffered many episodes of HUS, each treated with plasma exchange. In time, despite initiation of a prophylactic regimen of plasma exchange, his renal function declined significantly. At the age of 4 yr he received a (split liver) combined liver-kidney transplant (LKT) with preoperative plasma exchange and enoxaparin anticoagulation. Initial function of both grafts was excellent and is maintained for nearly 2 yr.
  
  
  CONCLUSIONS
  This report adds to the small but growing number of individuals in whom LKT has provided a favorable outcome for aHUS associated with CFH mutation, expands the technique of using a split liver graft, and describes the unique histologic features of subclinical liver disease in HUS.
authors.affiliation: Department of Pediatrics, The Mount Sinai Medical Center, One Gustave L Levy Place, New York, NY 10029, USA. Jeff.Saland@MSSM.EDU
authors.forename: Jeffrey M
authors.initials: JM
authors.lastname: Saland
authors.forename: Benjamin L
authors.initials: BL
authors.lastname: Shneider
authors.forename: Jonathan S
authors.initials: JS
authors.lastname: Bromberg
authors.forename: Patricia A
authors.initials: PA
authors.lastname: Shi
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Ward
authors.forename: Margret S
authors.initials: MS
authors.lastname: Magid
authors.forename: Corinne
authors.initials: C
authors.lastname: Benchimol
authors.forename: Mouin G
authors.initials: MG
authors.lastname: Seikaly
authors.forename: Sukru H
authors.initials: SH
authors.lastname: Emre
authors.forename: Elena
authors.initials: E
authors.lastname: Bresin
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Remuzzi
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D002675:Child, Preschool; D017242:Complement Factor H / Q000235:genetics*; D017984:Enoxaparin / Q000627:therapeutic use; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000235:genetics / Q000276:immunology / Q000601:surgery*; D006579:Heterozygote; D006801:Humans; D007676:Kidney Failure, Chronic / Q000235:genetics / Q000276:immunology / Q000517:prevention & control*; D016030:Kidney Transplantation; D016031:Liver Transplantation; D008297:Male; D009154:Mutation; D010951:Plasma Exchange; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D017984:Enoxaparin; D017242:Complement Factor H