Hemolytic uremic syndrome associated with Bordetella pertussis infection in a 2-month-old infant carrying a pathogenic variant in complement factor H.
Anti-Bacterial Agents
/ therapeutic use
Antibodies, Monoclonal, Humanized
/ therapeutic use
Atypical Hemolytic Uremic Syndrome
/ genetics
Binding Sites
/ genetics
Blood Transfusion
Bordetella pertussis
/ immunology
Complement Activation
/ genetics
Complement C3b
/ immunology
Complement Factor H
/ genetics
Humans
Infant
Infant, Newborn
Infant, Premature
Male
Peritoneal Dialysis
Polymorphism, Single Nucleotide
Whooping Cough
/ complications
Atypical HUS
Bordetella pertussis infection
Complement factor H
Eculizumab
Infant
Journal
Pediatric nephrology (Berlin, Germany)
ISSN: 1432-198X
Titre abrégé: Pediatr Nephrol
Pays: Germany
ID NLM: 8708728
Informations de publication
Date de publication:
03 2019
03 2019
Historique:
received:
10
05
2018
accepted:
10
12
2018
revised:
01
12
2018
pubmed:
19
12
2018
medline:
6
5
2020
entrez:
19
12
2018
Statut:
ppublish
Résumé
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.
Sections du résumé
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.
Identifiants
pubmed: 30560448
doi: 10.1007/s00467-018-4174-1
pii: 10.1007/s00467-018-4174-1
doi:
Substances chimiques
Anti-Bacterial Agents
0
Antibodies, Monoclonal, Humanized
0
Complement C3b
80295-43-8
Complement Factor H
80295-65-4
eculizumab
A3ULP0F556
Types de publication
Case Reports
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
533-537Références
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