Pediatric Nephrology

, Volume 34, Issue 3, pp 533–537 | Cite as

Hemolytic uremic syndrome associated with Bordetella pertussis infection in a 2-month-old infant carrying a pathogenic variant in complement factor H

  • Iona Madden
  • Lubka T. Roumenina
  • Hélène Langlois-Meurinne
  • Julie Guichoux
  • Brigitte Llanas
  • Véronique Frémeaux-Bacchi
  • Jérôme HarambatEmail author
  • Astrid Godron-Dubrasquet
Brief Report



Hemolytic uremic syndrome (HUS) has been associated with a number of infectious agents. We report here the case of an infant with severe Bordetella pertussis infection who developed HUS.

Case diagnosis/treatment

A 2-month-old preterm male was admitted for severe Bordetella pertussis infection. Symptoms leading to a diagnosis of hemolytic uremic syndrome (HUS) rapidly appeared: hemolytic anemia, thrombocytopenia, and acute kidney injury. He was treated with 25 days of peritoneal dialysis and received complement-targeting therapy with eculizumab (five injections over 2 months), in addition to blood transfusions, antibiotics, and respiratory support. The outcome was favorable. The genetic workup found a complement factor H gene variant which has been associated with atypical HUS. This variant was located in the C3b-binding site and functional tests revealed that it perturbed the regulatory activity of factor H.


This case suggests that pertussis is a strong trigger of HUS and that complement investigations are necessary to guide treatment and understand the pathophysiology.


Atypical HUS Complement factor H Bordetella pertussis infection Infant Eculizumab 


Funding source

The research was supported by grants from Agence Nationale de la Recherche ANR JCJC - INFLACOMP 2015-2018 ANR-15-CE15-0001 (to LTR), EU FP7 grant 2012-305608 (EURenOmics) (to VFB), APHP-PHRC AOM08198 (to VFB), and AIRG (to VFB), French Society of Nephrology (to VFB).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest

Supplementary material

467_2018_4174_MOESM1_ESM.docx (17 kb)
ESM 1 (DOCX 16.7 kb)
467_2018_4174_MOESM2_ESM.ppt (216 kb)
ESM 2 (PPT 215 kb)


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Copyright information

© IPNA 2018

Authors and Affiliations

  • Iona Madden
    • 1
    • 2
  • Lubka T. Roumenina
    • 3
    • 4
  • Hélène Langlois-Meurinne
    • 2
  • Julie Guichoux
    • 5
  • Brigitte Llanas
    • 1
  • Véronique Frémeaux-Bacchi
    • 3
    • 6
  • Jérôme Harambat
    • 1
    Email author
  • Astrid Godron-Dubrasquet
    • 1
  1. 1.Pediatric Nephrology UnitBordeaux University HospitalBordeauxFrance
  2. 2.Department of PediatricsBayonne Medical CentreBayonneFrance
  3. 3.INSERM, UMR_S 1138, Team “Complement and diseases”Cordeliers Research CenterParisFrance
  4. 4.Sorbonne Paris Cite, UMR_S 1138, Centre de Recherche des CordeliersUniversity Paris Descartes Paris 5ParisFrance
  5. 5.Pediatric Intensive Care UnitBordeaux University HospitalBordeauxFrance
  6. 6.Laboratory of ImmunologyGeorges Pompidou Hospital, AP-HPParisFrance

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