Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome.

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 re

SUBMITTER: Saland JM 

PROVIDER: S-EPMC2615708 | biostudies-literature | 2009 Jan

REPOSITORIES: biostudies-literature