Delayed Hematological Remission Predicts Poor Renal Outcome in Children with Atypical Hemolytic Uremic Syndrome

Background: Atypical hemolytic uremic syndrome (aHUS) is hemolytic uremic syndrome (HUS) without a coexisting disease or specific infection. Eculizumab is the standard of care for children with aHUS. However, since it is not yet available in India, plasma therapy remains the treatment of choice in these patients. We studied the clinical profile of children with aHUS and the determinants associated with low estimated glomerular filtration rate (eGFR) on follow-up.

Materials and methods: A retrospective chart review of children (1-18 years) with aHUS managed at a tertiary care center was done. Demographic details, clinical features, and investigations at presentation and on subsequent visits were noted. Details of treatment and duration of hospital stay were recorded.

Results: Of 26 children, boys outnumbered girls (2:1). The mean age at presentation was 80 ± 37.6 months. All children were hypertensive during the early phase of illness. Anti-factor H antibodies were elevated in 84% (22/26). Plasma therapy was initiated for 25 patients, and in 17 children, additionally immunosuppression was given. The median duration to achieve hematological remission was 17 days. As compared to children with normal eGFR, those with CKD stage 2 or more had significant delay in initiation of plasma therapy (4 vs. 14 days) and also took a longer time to achieve hematological remission (15 vs. 28 days). The prevalence of hypertension and proteinuria at the last follow-up was 63% and 27%, respectively.

Conclusion: Delayed initiation of plasma therapy and longer time to achieve hematological remission are associated with lower eGFR on follow-up. Long-term monitoring of hypertension and proteinuria is needed in these children.