Clinical features and management of atypical hemolytic uremic syndrome patient with DGKE gene variants: a case report.

This case suggest that atypical HUS with gene variant has a poor prognosis with a high mortality rate, which typically manifests in the first year of life and presents as a systemic disease with early-onset HUS with rapidly worsening renal function and chronic proteinuria. There is no specific treatment for -aHUS. There have an uncertain benefit of plasma therapy for -aHUS patients. The literature demonstrated that anti-complement therapy showed benefits for -aHUS with complement activation and autoantibodies during the overt TMA presentation but did not prevent TMA relapses. Early diagnosis and treatment may prevent complications and improve prognosis.