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

Madden, Iona; Roumenina, Lubka T; Langlois-Meurinne, Hélène; Guichoux, Julie; Llanas, Brigitte; Frémeaux-Bacchi, Véronique; Harambat, Jérôme; Godron-Dubrasquet, Astrid.
Pediatr Nephrol; 34(3): 533-537, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30560448


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.
Selo DaSilva