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J Infect Dis. 2017 Apr 15;215(8):1331-1338. doi: 10.1093/infdis/jix143.

Strains Responsible for Invasive Meningococcal Disease in Patients With Terminal Complement Pathway Deficiencies.

Author information

1
Laboratory of Immunology, Hôpital européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris (AP-HP).
2
Institut Pasteur, Invasive Bacterial Infection and National Reference Center for Meningococci.
3
Paris Descartes University, Sorbonne Paris Cité.
4
Department of Clinical Immunology, Hôpital Saint-Louis, AP-HP.
5
Institut National de la Santé et de la Recherche Médicale U1126, Hayem Center, Hôpital Saint-Louis, and.
6
Immuno-Hematology Unit, Hôpital Robert Debré, AP-HP, Paris.
7
Pediatric Hematology-Oncology Unit, Hôpital Mère Enfant, Hôpital de Nantes.
8
University of Lille, U995-LIRIC, Lille Inflammation Research International Center.
9
Institut National de la Santé et de la Recherche Médicale, U995.
10
Departement of Internal Medicine and Clinical Immunology, Hôpital de Lille, and.
11
Centre National de Référence des Maladies Autoimmunes et Systémiques Rares (Sclérodermie), Lille.
12
Pediatric Department, Hôpitaux universitaires Paris Seine Saint Denis, AP-HP, Paris 13 University.
13
Hematology Departement, Hôpital Necker-Enfants malades, AP-HP.
14
Institut National de la Santé et de la Recherche Médicale UMR1163, Imagine Institute, Paris Descartes University.
15
Department of Pediatric Immunology, Hematology, and Rheumatology and.
16
Study Center for Primary Immunodeficiencies, Hôpital Necker-Enfants malades, AP-HP, and.
17
Institut National de la Santé et de la Recherche Médicale UMRS 1138, Cordeliers Research Center, Paris, France.

Abstract

Background:

Patients with terminal complement pathway deficiency (TPD) are susceptible to recurrent invasive meningococcal disease (IMD). Neisseria meningitidis (Nm) strains infecting these patients are poorly documented in the literature.

Methods:

We identified patients with TPD and available Nm strains isolated during IMD. We investigated the genetic basis of the different TPDs and the characteristics of the Nm strains.

Results:

We included 56 patients with C5 (n = 8), C6 (n = 20), C7 (n = 18), C8 (n = 9), or C9 (n = 1) deficiency. Genetic study was performed in 47 patients and 30 pathogenic variants were identified in the genes coding for C5 (n = 4), C6 (n = 5), C7 (n = 12), C8 (n = 7), and C9 (n = 2). We characterized 61 Nm strains responsible for IMD in the 56 patients with TPD. The most frequent strains belonged to groups Y (n = 27 [44%]), B (n = 18 [30%]), and W (n = 8 [13%]). Hyperinvasive clonal complexes (CC11, CC32, CC41/44, and CC269) were responsible for 21% of IMD cases. The CC23 predominates and represented 26% of all invasive isolates. Eleven of the 15 clonal complexes identified fit to 12 different clonal complexes belonging to carriage strains.

Conclusions:

Unusual meningococcal strains with low level of virulence similar to carriage strains are most frequently responsible for IMD in patients with TPD.

KEYWORDS:

Neisseria meningitidis; complement; membrane attack complex.; primary immunodeficiency; terminal complement pathway

PMID:
28368462
DOI:
10.1093/infdis/jix143
[Indexed for MEDLINE]
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