Postoperative Atypical Hemolytic Uremic Syndrome Associated with Complement C3 Mutation
Joint Authors
Matsukuma, Eiji
Imamura, Atsushi
Iwata, Yusuke
Takeuchi, Takamasa
Yoshida, Yoko
Fujimura, Yoshihiro
Fan, Xinping
Kuwahara, Takashi
Miyata, T.
Source
Issue
Vol. 2014, Issue 2014 (31 Dec. 2014), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2014-11-06
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
Atypical hemolytic uremic syndrome (aHUS) can be distinguished from typical or Shiga-like toxin-induced HUS.
The clinical outcome is unfavorable; up to 50% of affected patients progress to end-stage renal failure and 25% die during the acute phase.
Multiple conditions have been associated with aHUS, including infections, drugs, autoimmune conditions, transplantation, pregnancy, and metabolic conditions.
aHUS in the nontransplant postsurgical period, however, is rare.
An 8-month-old boy underwent surgical repair of tetralogy of Fallot.
Neurological disturbances, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia developed 25 days later, and aHUS was diagnosed.
Further evaluation revealed that his complement factor H (CFH) level was normal and that anti-FH antibodies were not detected in his plasma.
Sequencing of his CFH, complement factor I, membrane cofactor protein, complement factor B, and thrombomodulin genes was normal.
His ADAMTS-13 (a disintegrin-like and metalloprotease with thrombospondin-1 repeats 13) activity was also normal.
However, he had a potentially causative mutation (R425C) in complement component C3.
Restriction fragment length polymorphism analysis revealed that his father and aunt also had this mutation; however, they had no symptoms of aHUS.
We herein report a case of aHUS that developed after cardiovascular surgery and was caused by a complement C3 mutation.
American Psychological Association (APA)
Matsukuma, Eiji& Imamura, Atsushi& Iwata, Yusuke& Takeuchi, Takamasa& Yoshida, Yoko& Fujimura, Yoshihiro…[et al.]. 2014. Postoperative Atypical Hemolytic Uremic Syndrome Associated with Complement C3 Mutation. Case Reports in Nephrology،Vol. 2014, no. 2014, pp.1-5.
https://search.emarefa.net/detail/BIM-1034811
Modern Language Association (MLA)
Matsukuma, Eiji…[et al.]. Postoperative Atypical Hemolytic Uremic Syndrome Associated with Complement C3 Mutation. Case Reports in Nephrology No. 2014 (2014), pp.1-5.
https://search.emarefa.net/detail/BIM-1034811
American Medical Association (AMA)
Matsukuma, Eiji& Imamura, Atsushi& Iwata, Yusuke& Takeuchi, Takamasa& Yoshida, Yoko& Fujimura, Yoshihiro…[et al.]. Postoperative Atypical Hemolytic Uremic Syndrome Associated with Complement C3 Mutation. Case Reports in Nephrology. 2014. Vol. 2014, no. 2014, pp.1-5.
https://search.emarefa.net/detail/BIM-1034811
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1034811