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Byline: Hae Il Cheong (1,4), Byong Sop Lee (1), Hee-Gyung Kang (1), Hyewon Hahn (2), Kyung-Suk Suh (3), Il Soo Ha (1), Yong Choi (1) Keywords: Factor H deficiency; Complement; Hemolytic uremic syndrome; Auxiliary partial orthotopic liver transplantation Abstract: Complement factor H (FH) deficiency is one of the causes of atypical hemolytic uremic syndrome (HUS). Most patients with FH deficiency associated HUS progress to end-stage renal disease despite plasma therapy. Moreover, the disease invariably recurs in the graft kidney and causes graft failure. We confirmed FH deficiency in a 30-month-old boy with recurrent HUS of 2 years duration, and attempted an auxiliary partial orthotopic liver transplantation (APOLT) to overcome the sustained intractable dependency on plasma therapy. APOLT restored the plasma FH level, without HUS recurrence, for 7 months. However, thereafter he suffered from serious infectious complications associated with immunosuppression and finally died 11 months after APOLT. In conclusion, although APOLT showed clinical and laboratory improvement for some period in this patient, the final fatal outcome suggests that liver transplantation should be cautiously applied to patients with HUS associated with FH deficiency. Author Affiliation: (1) Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea (2) Department of Pediatrics, Ulsan Medical School, Asan Medical Center, Seoul, Korea (3) Department of General Surgery, Seoul National University Hospital, Seoul, Korea (4) Department of Pediatrics, Seoul National University Children's Hospital, 28 Yongon-dong, Chongro-gu, Seoul 110--744, Korea Article History: Registration Date: 11/11/2003 Received Date: 20/06/2003 Accepted Date: 03/11/2003 Online Date: 24/02/2004