RESUMO
Atypical hemolytic uremic syndrome (aHUS) is a rare acute and critical disease in childhood, which is easy to lead to acute kidney injury, and has a high mortality rate and chronic kidney disease incidence if not diagnosed and treated in time.According to the etiology, aHUS can be classified into hereditary and acquired.Anti-factor H antibody associated aHUS is acquired and mainly occurs in children aged 5 to 15 years.Anti-factor H antibody associated aHUS is strongly associated with homozygous deletion of the complement H related protein 1/3 (CFRH1/3) gene.In recent years, there have been significant advances in the etiology, genetics, and immunology of aHUS, especially the treatment of Eculizumab, which has greatly improved the prognosis of the disease.However, at present, there are still problems that need to be solved in the pathogenesis, diagnosis, treatment and prognosis of antibody-related aHUS, and this article will discuss the above content and put forward corresponding prospects to provide reference for clinical and scientific research.