Severe infantile Bordetella pertussis pneumonia in monozygotic twins with a congenital C3 deficiency
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Publication year
2014Source
European Journal of Pediatrics, 173, 12, (2014), pp. 1591-4ISSN
Publication type
Article / Letter to editor
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Organization
Paediatrics - OUD tm 2017
Laboratory Medicine
Journal title
European Journal of Pediatrics
Volume
vol. 173
Issue
iss. 12
Page start
p. 1591
Page end
p. 4
Subject
Radboudumc 0: Other Research RIHS: Radboud Institute for Health Sciences; Radboudumc 11: Renal disorders RIMLS: Radboud Institute for Molecular Life SciencesAbstract
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 C3 mutation 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.
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- Academic publications [238441]
- Electronic publications [122520]
- Faculty of Medical Sciences [90373]
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