Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome.
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.
Saland
Jeffrey M
JM
Department of Pediatrics, The Mount Sinai Medical Center, One Gustave L Levy Place, New York, NY 10029, USA. Jeff.Saland@MSSM.EDU
Shneider
Benjamin L
BL
Bromberg
Jonathan S
JS
Shi
Patricia A
PA
Ward
Stephen C
SC
Magid
Margret S
MS
Benchimol
Corinne
C
Seikaly
Mouin G
MG
Emre
Sukru H
SH
Bresin
Elena
E
Remuzzi
Giuseppe
G
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
D000925:Anticoagulants; D017984:Enoxaparin; D017242:Complement Factor H