Kidney Transplantation in Patients with Atypical Hemolytic Uremic Syndrome due to Complement Factor H Deficiency: Impact of Liver Transplantation
- Author:
Sejin KIM
1
;
Eujin PARK
;
Sang il MIN
;
Nam Joon YI
;
Jongwon HA
;
Il Soo HA
;
Hae Il CHEONG
;
Hee Gyung KANG
Author Information
- Publication Type:Original Article
- Keywords: Atypical Hemolytic Uremic Syndrome; Complement Factor H, Kidney Transplantation; Liver Transplantation
- MeSH: Allografts; Atypical Hemolytic Uremic Syndrome; Complement Factor H; Complement System Proteins; Humans; Kidney Failure, Chronic; Kidney Transplantation; Kidney; Liver Transplantation; Liver; Plasma; Rare Diseases; Recurrence
- From:Journal of Korean Medical Science 2018;33(1):e4-
- CountryRepublic of Korea
- Language:English
- Abstract: BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disease that is often associated with genetic defects. Mutations of complement factor H (CFH) are the most common genetic defects that cause aHUS and often result in end-stage renal disease. Since CFH is mainly produced in the liver, liver transplantation (LT) has been performed in patients with defective CFH. METHODS: The clinical courses of four kidney allograft recipients who lost their native kidney functions due to aHUS associated with a CFH mutation were reviewed. RESULTS: Subject A underwent kidney transplantation (KT) twice, aHUS recurred and the allograft kidney failed within a few years. Subject B received a KT and soon experienced a recurrence of aHUS coinciding with infection. Her allograft kidney function has worsened, and she remains on plasma infusion therapy. Subject C underwent LT followed by KT. She is doing well without plasma infusion therapy after combined LT-KT for 3 years. Subject D received KT following LT and is now recurrence-free from aHUS. CONCLUSION: In patients with aHUS associated with a CFH mutation, KT without LT was complicated with a recurrence of aHUS, which might lead to allograft loss. Conversely, LT was successful in preventing the recurrence of aHUS and thus might be another option for a recurrence-free life for aHUS patients associated with CFH mutation.
