CM-HUS: complement-mediated hemolytic uremic syndrome; RBC: red blood cell; LDH: lactate dehydrogenase; MAHA: microangiopathic hemolytic anemia; AKI: acute kidney injury; TTP: thrombotic thrombocytopenic purpura; CFH: complement factor H; VUS: variant of uncertain significance.
* Details of diagnosis are discussed in UpToDate; we generally consider the following to be supportive of a diagnosis of CM-HUS:¶ If TTP remains a possibility (results of ADAMTS13 activity pending), therapeutic plasma exchange (TPE) may also be appropriate. Anti-complement therapy should be given immediately following each TPE procedure so that the TPE does not remove the complement blocking monoclonal antibody. Eculizumab and ravulizumab are both complement-blocking monoclonal antibodies; they are equally effective as anti-complement therapy and differ only in the dosing interval.
Δ Refer to UpToDate for details of genetic and serologic testing. Genetic test results may take weeks to months to return. Pathogenic sequence variants in the 5 genes listed above are the most clinically important in CM-HUS. Variants in all other genes such as CFHR or THBD typically are not pathogenic. If a sequence variant of uncertain significance (VUS) is identified, manage according to the personal and family history and continue to review information about pathogenicity (from the testing laboratory, complement expert, or a genetics resource such as ClinVar).آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟