TY - JOUR
T1 - Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome
AU - Saland, Jeffrey M.
AU - Shneider, Benjamin L.
AU - Bromberg, Jonathan S.
AU - Shi, Patricia A.
AU - Ward, Stephen C.
AU - Magid, Margret S.
AU - Benchimol, Corinne
AU - Seikaly, Mouin G.
AU - Emre, Sukru H.
AU - Bresin, Elena
AU - Remuzzi, Giuseppe
PY - 2009/1/1
Y1 - 2009/1/1
N2 - 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.
AB - 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.
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U2 - 10.2215/CJN.02170508
DO - 10.2215/CJN.02170508
M3 - Article
C2 - 19005013
AN - SCOPUS:64049101602
SN - 1555-9041
VL - 4
SP - 201
EP - 206
JO - Clinical Journal of the American Society of Nephrology
JF - Clinical Journal of the American Society of Nephrology
IS - 1
ER -