Premium
Autoimmune‐type atypical hemolytic uremic syndrome treated with eculizumab as first‐line therapy
Author(s) -
Hisano Masataka,
Ashida Akira,
Nakano Eiji,
Suehiro Mamiko,
Yoshida Yoko,
Matsumoto Masanori,
Miyata Toshiyuki,
Fujimura Yoshihiro,
Hattori Motoshi
Publication year - 2015
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12469
Subject(s) - medicine , atypical hemolytic uremic syndrome , eculizumab , microangiopathic hemolytic anemia , oliguria , thrombotic microangiopathy , hemolytic anemia , hemolysis , dialysis , acute kidney injury , gastroenterology , complement system , immunology , thrombotic thrombocytopenic purpura , antibody , renal function , platelet , disease
We report a case of atypical hemolytic uremic syndrome ( aHUS ) in a 4‐year‐old boy. Although the patient had the typical triad of aHUS (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury), urgent dialysis was not indicated because he had neither oliguria nor severe electrolyte abnormality. He was given eculizumab as first‐line therapy, which led to significant clinical improvement, thus avoiding any risk of complications associated with plasma exchange and central venous catheterization. Retrograde functional analysis of the patient's plasma using sheep erythrocytes indicated an increase in hemolysis, suggesting impairment of host cell protection by complement factor H . The use of eculizumab as first‐line therapy in place of plasma exchange might be reasonable for pediatric patients with aHUS .