Hemolytic uremic syndrome associated with Bordetella pertussis infection in a 2-month-old infant carrying a pathogenic variant in complement factor H
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.
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.
KeywordsAtypical HUS Complement factor H Bordetella pertussis infection Infant Eculizumab
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).
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Conflict of interest
The authors declare that they have no conflict of interest
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