Dataset Information
Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome.
Ontology highlight
ABSTRACT: Background and objectives
A male infant with a family history of thrombotic microangiopathy developed atypical hemolytic uremic syndrome (aHUS).Design, setting, participants, & measurements
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.
SUBMITTER: Saland JM
PROVIDER: S-EPMC2615708 | biostudies-literature | 2009 Jan
REPOSITORIES: biostudies-literature
Publications
Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome.
Clinical journal of the American Society of Nephrology : CJASN 20081112 1
<h4>Background and objectives</h4>A male infant with a family history of thrombotic microangiopathy developed atypical hemolytic uremic syndrome (aHUS).<h4>Design, setting, participants, & measurements</h4>Case report.<h4>Results</h4>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, hi ...[more]