Prognosis

Shiga toxin-producing Escherichia coli (STEC) HUS

Mortality associated with STEC HUS is reported to be approximately 3%.[81] Patients presenting with the highest leukocyte counts (>25,000 cells/microlitre) are at greatest risk of death.[81]

The typical duration of dialysis in affected children is about 1 to 2 weeks. The incidence of neurological involvement in the form of seizures, coma, stroke, and/or altered mental status is reported to be around 17% to 34%.[61][65][82]

Long-term renal damage may present as chronic renal insufficiency, hypertension, and/or proteinuria. It is important to screen patients for hypertension and proteinuria on follow-up visits into adulthood. In children with duration of dialysis longer than 2 weeks, it is prudent to monitor renal function over time.

Atypical HUS and secondary HUS

Because patients with this diagnosis are generally grouped together with thrombotic thrombocytopenic purpura (TTP) in case series, it is difficult to estimate prognosis with any accuracy.

Eculizumab has revolutionised outcomes for patients with atypical HUS (aHUS). Both mortality rates and the rate of end-stage renal disease have declined in these patients.[5][70][71][73][74]

Children with Streptococcus pneumoniae-related HUS have a more prolonged hospital course and more frequently require dialysis, but they have a high likelihood of recovery of renal function.[83]

The prognosis for patients with HUS secondary to bone marrow transplant and chemotherapy agents is poor.[84][85][86] Reports of patients with thrombotic microangiopathy associated with the use of targeted cancer agents (e.g., immunotoxins, monoclonal antibodies, and tyrosine kinase inhibitors) suggest that outcomes are better than in people with chemotherapy-associated thrombotic microangiopathy, although further research is needed.[19]

Familial HUS

Progression to acute kidney injury is common.[17][87][88] Treatment with plasma exchange has been reported to prevent acute kidney injury in rare cases.[53][89] Eculizumab has shown clinical activity, but long-term results are not available.[73]

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