Relapsing Campylobacter jejuni Systemic Infections in a Child with X-Linked Agammaglobulinemia

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency of the humoral compartment, due to a mutation in the Bruton tyrosine kinase (BTK) gene, characterized by a severe defect of circulating B cells and serum immunoglobulins. Recurrent infections are the main clinical manifestations; althou...

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Main Authors: Paola Ariganello, Giulia Angelino, Alessia Scarselli, Irene Salfa, Martina Della Corte, Arianna De Matteis, Patrizia D'Argenio, Susanna Livadiotti, Emma C. Manno, Cristina Russo, Andrea Finocchi, Caterina Cancrini
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Pediatrics
Online Access:http://dx.doi.org/10.1155/2013/735108
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author Paola Ariganello
Giulia Angelino
Alessia Scarselli
Irene Salfa
Martina Della Corte
Arianna De Matteis
Patrizia D'Argenio
Susanna Livadiotti
Emma C. Manno
Cristina Russo
Andrea Finocchi
Caterina Cancrini
author_facet Paola Ariganello
Giulia Angelino
Alessia Scarselli
Irene Salfa
Martina Della Corte
Arianna De Matteis
Patrizia D'Argenio
Susanna Livadiotti
Emma C. Manno
Cristina Russo
Andrea Finocchi
Caterina Cancrini
author_sort Paola Ariganello
collection DOAJ
description X-linked agammaglobulinemia (XLA) is a primary immunodeficiency of the humoral compartment, due to a mutation in the Bruton tyrosine kinase (BTK) gene, characterized by a severe defect of circulating B cells and serum immunoglobulins. Recurrent infections are the main clinical manifestations; although they are especially due to encapsulated bacteria, a specific association with Campylobacter species has been reported. Here, we report the case of a boy with XLA who presented with relapsing Campylobacter jejuni systemic infections. His clinical history supports the hypothesis of the persistence of C. jejuni in his intestinal tract. Indeed, as previously reported, XLA patients may become chronic intestinal carriers of Campylobacter, even in absence of symptoms, with an increased risk of relapsing bacteraemia. The humoral defect is considered to be crucial for this phenomenon, as well as the difficulties to eradicate the pathogen with an appropriate antibiotic therapy; drug resistance is raising in Campylobacter species, and the appropriate duration of treatment has not been established. C. jejuni should always be suspected in XLA patients with signs and symptoms of systemic infection, and treatment should be based on antibiogram to assure the eradication of the pathogen.
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spelling doaj-art-68813fd36ca1419694b8b780abd3585a2025-02-03T01:32:48ZengWileyCase Reports in Pediatrics2090-68032090-68112013-01-01201310.1155/2013/735108735108Relapsing Campylobacter jejuni Systemic Infections in a Child with X-Linked AgammaglobulinemiaPaola Ariganello0Giulia Angelino1Alessia Scarselli2Irene Salfa3Martina Della Corte4Arianna De Matteis5Patrizia D'Argenio6Susanna Livadiotti7Emma C. Manno8Cristina Russo9Andrea Finocchi10Caterina Cancrini11University-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyDepartment of Laboratories, Unit of Microbiology, Children’s Hospital Bambino Gesù, Piazza Sant'Onofrio, 4-00165 Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyUniversity-Hospital Department of Pediatrics, Unit of Immunology and Infectious Disease, Children’s Hospital Bambino Gesù and University of Rome “Tor Vergata”, Rome, ItalyX-linked agammaglobulinemia (XLA) is a primary immunodeficiency of the humoral compartment, due to a mutation in the Bruton tyrosine kinase (BTK) gene, characterized by a severe defect of circulating B cells and serum immunoglobulins. Recurrent infections are the main clinical manifestations; although they are especially due to encapsulated bacteria, a specific association with Campylobacter species has been reported. Here, we report the case of a boy with XLA who presented with relapsing Campylobacter jejuni systemic infections. His clinical history supports the hypothesis of the persistence of C. jejuni in his intestinal tract. Indeed, as previously reported, XLA patients may become chronic intestinal carriers of Campylobacter, even in absence of symptoms, with an increased risk of relapsing bacteraemia. The humoral defect is considered to be crucial for this phenomenon, as well as the difficulties to eradicate the pathogen with an appropriate antibiotic therapy; drug resistance is raising in Campylobacter species, and the appropriate duration of treatment has not been established. C. jejuni should always be suspected in XLA patients with signs and symptoms of systemic infection, and treatment should be based on antibiogram to assure the eradication of the pathogen.http://dx.doi.org/10.1155/2013/735108
spellingShingle Paola Ariganello
Giulia Angelino
Alessia Scarselli
Irene Salfa
Martina Della Corte
Arianna De Matteis
Patrizia D'Argenio
Susanna Livadiotti
Emma C. Manno
Cristina Russo
Andrea Finocchi
Caterina Cancrini
Relapsing Campylobacter jejuni Systemic Infections in a Child with X-Linked Agammaglobulinemia
Case Reports in Pediatrics
title Relapsing Campylobacter jejuni Systemic Infections in a Child with X-Linked Agammaglobulinemia
title_full Relapsing Campylobacter jejuni Systemic Infections in a Child with X-Linked Agammaglobulinemia
title_fullStr Relapsing Campylobacter jejuni Systemic Infections in a Child with X-Linked Agammaglobulinemia
title_full_unstemmed Relapsing Campylobacter jejuni Systemic Infections in a Child with X-Linked Agammaglobulinemia
title_short Relapsing Campylobacter jejuni Systemic Infections in a Child with X-Linked Agammaglobulinemia
title_sort relapsing campylobacter jejuni systemic infections in a child with x linked agammaglobulinemia
url http://dx.doi.org/10.1155/2013/735108
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