Hemolytic uremic syndrome associated with Bordetella pertussis infection in a 2-month-old infant carrying a pathogenic variant in complement factor H.
Pediatr Nephrol
; 34(3): 533-537, 2019 03.
Article
in En
| MEDLINE
| ID: mdl-30560448
ABSTRACT
BACKGROUND:
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.CONCLUSION:
This case suggests that pertussis is a strong trigger of HUS and that complement investigations are necessary to guide treatment and understand the pathophysiology.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Bordetella pertussis
/
Complement C3b
/
Whooping Cough
/
Complement Factor H
/
Atypical Hemolytic Uremic Syndrome
Type of study:
Risk_factors_studies
Limits:
Humans
/
Infant
/
Male
/
Newborn
Language:
En
Journal:
Pediatr Nephrol
Journal subject:
NEFROLOGIA
/
PEDIATRIA
Year:
2019
Document type:
Article
Affiliation country:
France