Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treated with the complement inhibitor eculizumab. Eculizumab was shown to completely inhibit haemolysis, normalize thrombocyte levels and increase diuresis. Full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation. These findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy, in order to avoid irreversible renal damage.
机构:
Tokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Terano, Chikako
Ishikura, Kenji
论文数: 0引用数: 0
h-index: 0
机构:
Tokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Natl Ctr Child Hlth & Dev, Div Nephrol & Rheumatol, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Ishikura, Kenji
Hamada, Riku
论文数: 0引用数: 0
h-index: 0
机构:
Tokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Hamada, Riku
Yoshida, Yasuhiro
论文数: 0引用数: 0
h-index: 0
机构:
Univ Tokyo, Grad Sch Med, Dept Pediat, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Yoshida, Yasuhiro
Kubota, Wataru
论文数: 0引用数: 0
h-index: 0
机构:
Tokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Kubota, Wataru
Okuda, Yusuke
论文数: 0引用数: 0
h-index: 0
机构:
Shiga Univ Med Sci, Dept Pediat, Otsu, Shiga, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Okuda, Yusuke
Shinozuka, Shunsuke
论文数: 0引用数: 0
h-index: 0
机构:
Natl Hosp Org Saitama Natl Hosp, Dept Pediat, Saitama, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Shinozuka, Shunsuke
Harada, Ryoko
论文数: 0引用数: 0
h-index: 0
机构:
Tokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Harada, Ryoko
Iyoda, Sunao
论文数: 0引用数: 0
h-index: 0
机构:
Natl Inst Infect Dis, Dept Bacteriol 1, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Iyoda, Sunao
Fujimura, Yoshihiro
论文数: 0引用数: 0
h-index: 0
机构:
Nara Med Univ, Dept Blood Transfus Med, Kashihara, Nara, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Fujimura, Yoshihiro
Hamasaki, Yuko
论文数: 0引用数: 0
h-index: 0
机构:
Tokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Toho Univ, Dept Pediat Nephrol, Fac Med, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Hamasaki, Yuko
Hataya, Hiroshi
论文数: 0引用数: 0
h-index: 0
机构:
Tokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan
Hataya, Hiroshi
Honda, Masataka
论文数: 0引用数: 0
h-index: 0
机构:
Tokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, JapanTokyo Metropolitan Childrens Med Ctr, Dept Nephrol, Fuchu, Tokyo, Japan