Severe infantile Bordetella pertussis pneumonia in monozygotic twins with a congenital C3 deficiency

Roel A. J. Kurvers, Dineke Westra, Arno F. van Heijst, Twiggy L. M. Walk, Adilia Warris, Nicole C. A. J. van de Kar

Research output: Contribution to journalArticlepeer-review

8 Citations (Scopus)


Bordetella pertussis or whooping cough is a vaccine-preventable disease that still remains a serious infection in neonates and young infants. We describe two young infants, monozygotic twins, with a severe B. pertussis pneumonia of whom one needed extracorporeal membrane oxygenation. Diagnostic work-up of unexplained hematuria and proteinuria during the illness revealed low serum complement component 3 (C3) levels. During follow-up, C3 levels remained low (400–600 mg/L). Extensive analysis of the persistent low C3 levels revealed an unknown heterozygous mutation in the C3 gene in both siblings and their mother. This C3mutation in combination with the specific virulence mechanisms of B. pertussis probably contributed to the severe disease course in these cases. Conclusion: We propose that genetically caused complement disorders should be considered when confronted with severe cases of B. pertussis infection.

Original languageEnglish
Pages (from-to)1591-1594
Number of pages4
JournalEuropean Journal of Pediatrics
Issue number12
Early online date21 Aug 2013
Publication statusPublished - Dec 2014

Bibliographical note

D.W. is supported by the Dutch Kidney Foundation (C09.2313).

Conflict of interest
The authors have no conflicts of interest to declare.


  • Complement C3
  • Bordetella pertussis
  • infant
  • complement
  • complement inhibitors


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