Article
Analysis of patients with atypical hemolytic uremic syndrome treated at the Mie University Hospital: concentration of C3 p.I1157T mutation.
International journal of hematology - 1 Nov 2014
Matsumoto Takeshi, Fan Xinping, Ishikawa Eiji, Ito Masaaki, Amano Keishirou, Toyoda Hidemi, Komada Yoshihiro, Ohishi Kohshi, Katayama Naoyuki, Yoshida Yoko, Matsumoto Masanori, Fujimura Yoshihiro, Ikejiri Makoto, Wada Hideo, Miyata Toshiyuki
Abstract excerpt
Atypical hemolytic uremic syndrome (aHUS) is caused by abnormalities of the complement system and has a significantly poor prognosis. The clinical phenotypes of 12 patients in nine families with aHUS with familial or recurrent onset and ADAMTS13 activity of ≥20 % treated at the Mie University Hospital were examined. In seven of the patients, the first episode of aHUS occurred during childhood and ten patients...
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