Eculizumab use in patients with pneumococcal-associated hemolytic uremic syndrome and kidney outcomes

Background Streptococcus pneumoniae -associated hemolytic uremic syndrome (P-HUS) is a rare and severe disease. Only a few reports have been published about eculizumab use in P-HUS. Methods We analyzed demographic, clinical, and laboratory data of patients with P-HUS from our center. Results The coh...

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Veröffentlicht in:Pediatric nephrology (Berlin, West) West), 2023-12, Vol.38 (12), p.4209-4215
Hauptverfasser: Konopásek, Patrik, Zieg, Jakub
Format: Artikel
Sprache:eng
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Zusammenfassung:Background Streptococcus pneumoniae -associated hemolytic uremic syndrome (P-HUS) is a rare and severe disease. Only a few reports have been published about eculizumab use in P-HUS. Methods We analyzed demographic, clinical, and laboratory data of patients with P-HUS from our center. Results The cohort consisted of 4 females and 3 males. All patients had pneumonia. Four were given eculizumab (days 1–3). The eculizumab group required a shorter duration of dialysis and mechanical ventilation (medians 20 vs. 28.5 and 30 vs 38.5 days, respectively) compared with the non-eculizumab group, but this was still much longer than normally reported; the thrombocytopenia resolution was similar in both groups (medians 10 vs. 8 days). Chronic kidney disease (CKD) was correlated with the duration of dialysis and mechanical ventilation duration at 1 year ( r  = 0.797, P  = 0.032 and r  = 0.765, P  = 0.045) and last follow-up ( r  = 0.807, P  = 0.028 and r  = 0.814, P  = 0.026, respectively); our scoring system showed even stronger correlations ( r  = 0.872, P  = 0.011 and r  = 0.901, P  = 0.0057, respectively). The eculizumab group showed slightly better 1-year and last follow-up CKD stage (2.75 vs. 3, P  = 0.879 and 2.5 vs. 3.67, P  = 0.517). Conclusions Despite the fact that the eculizumab group showed better outcomes, eculizumab does not seem to improve the course of P-HUS compared with previous reports. Kidney outcomes are strongly correlated with the duration of dialysis and mechanical ventilation duration. Graphical Abstract A higher resolution version of the Graphical abstract is available as Supplementary information
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-023-06037-2