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


Journal

Pediatric nephrology (Berlin, Germany)
ISSN: 1432-198X
Titre abrégé: Pediatr Nephrol
Pays: Germany
ID NLM: 8708728

Informations de publication

Date de publication:
12 2023
Historique:
received: 27 03 2023
accepted: 23 05 2023
revised: 05 05 2023
medline: 23 10 2023
pubmed: 12 6 2023
entrez: 12 6 2023
Statut: ppublish

Résumé

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. We analyzed demographic, clinical, and laboratory data of patients with P-HUS from our center. 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). 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. A higher resolution version of the Graphical abstract is available as Supplementary information.

Sections du résumé

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. A higher resolution version of the Graphical abstract is available as Supplementary information.

Identifiants

pubmed: 37306721
doi: 10.1007/s00467-023-06037-2
pii: 10.1007/s00467-023-06037-2
pmc: PMC10584715
doi:

Substances chimiques

eculizumab A3ULP0F556

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

4209-4215

Informations de copyright

© 2023. The Author(s).

Références

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Auteurs

Patrik Konopásek (P)

Department of Pediatric Nephrology, 2nd Faculty of Medicine, University Hospital Motol, Charles University, Prague, Czech Republic. pat.konopasek@gmail.com.
Pediatrická Klinika 2. LF UK and FN v Motole, V Úvalu 84, Prague 5, 15006, Czech Republic. pat.konopasek@gmail.com.

Jakub Zieg (J)

Department of Pediatric Nephrology, 2nd Faculty of Medicine, University Hospital Motol, Charles University, Prague, Czech Republic.

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