Vaccination against encapsulated bacteria in hereditary C2 deficiency results in antibody response and opsonization due to antibody-dependent complement activation

Jönsson, Göran; Lood, Christian; Gullstrand, Birgitta; Holmström, Eva; Selander, Barbro; Braconier, Jean Henrik; Sturfelt, Gunnar; Bengtsson, Anders; Truedsson, Lennart

doi:10.1016/j.clim.2012.06.008

Cited by 23 publications

(13 citation statements)

References 56 publications

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“…The defects in the complement system are in general not possible to correct, but many complications are avoidable if the defect is identified early enough. In C2 deficiency, vaccination against the encapsulated bacteria S. pneumoniae and H. influenzae can give rise to antibodies and opsonisation, clearly suggesting increased protection [36]. Vaccination against other encapsulated bacteria such as meningococci is also recommended in complement deficiency states.…”

Section: Discussionmentioning

confidence: 99%

Primary immunodeficiency in infection-prone children in southern Sweden: occurrence, clinical characteristics and immunological findings

et al. 2014

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BackgroundPrimary immunodeficiency diseases (PIDs) comprise a heterogeneous group of disorders mainly characterized by increased susceptibility to infections. The aims of this study were to estimate the occurrence rate of PID in the paediatric (age ≤ 18 years) population of southern Sweden (approx. 265,000 children) and to describe their demographic, clinical and immunological characteristics. During a period of 4 years, in four paediatric speciality clinics in Skåne County in southern Sweden, children being seen for infections and fulfilling specific criteria were evaluated according to a predefined examination schedule. The initial analysis consisted of complete blood counts with analysis of lymphocyte subpopulations (T, B, NK cells), measurement of immunoglobulins (IgG, IgA, IgM, IgE and IgG subclasses), and assessment of the complement system (classical, alternative and lectin pathways). In addition, results of these immunological analyses in other children from the same area and time period were evaluated.ResultsIn total, 259 children (53.6% males) met the criteria and were included. The most common infection was recurrent otitis media. Immunological analyses results for about two thirds of the patients were outside age-related reference intervals. Further examination in this latter group identified 15 children with PID (9 males); 7 (2.7%) had genetically defined PID, representing 4 different diagnoses, and another 8 (3.1%) had a clinically defined PID - common variable immunodeficiency. No additional PID patient was identified from the evaluation of laboratory results in children not included in the study. The median age at diagnosis was 3.5 years (range 1–12 years).ConclusionsThe occurrence rate of PID was about 4 new cases per year in this population. Several different PID diagnoses were found, and the application of specified criteria to identify PID patients was useful. In children who are prone to infection, the use of a predefined set of immunological laboratory analyses at their first examination was beneficial for early identification of patients with PID.

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Section: Discussionmentioning

confidence: 99%

Primary immunodeficiency in infection-prone children in southern Sweden: occurrence, clinical characteristics and immunological findings

et al. 2014

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“…As a preventive measure, revaccination to Pneumococcus and to Meningococcus was achieved, in agreement with Jönsson et al (2012). [17] Additional perplexities on the indication to the CI in the reported boy derived from his clinical history: the post-meningitic nature of deafness and the long period of partial hearing deprivation (9 years).…”

Section: Discussionmentioning

confidence: 99%

“…At the light of new relevant knowledge about C2D [16,17] and after a new multidisciplinary evaluation, CI was considered feasible, provided that the IgG and IgG subclass levels were normal and that protective levels of antibodies against Pneumococci, Haemophilus Influentiae and…”

Section: Case Reportmentioning

confidence: 99%

Delayed cochlear implantation in post-meningitic deafness and hereditary complement C2 deficiency

Berardino

Truedsson

Zanetti

2017

International Journal of Pediatric Otorhinolaryngology Extra

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We report the case of an adolescent with post-verbal severe/profound sensorineural hearing loss, occurring as a consequence of two bouts of pneumococcal meningitis at 12 and 32 months of age. A possible immunodeficiency was investigated, revealing hereditary complement C2 deficiency (C2D). Given the insufficient benefit from high-power hearing aids, the boy received a cochlear implant (CI) at age 12. Despite the long interval of partial hearing deprivation and the postmeningitic etiology, improvement in open-set speech perception and quality of life were observed.The C2D did not favour post-operative infections nor meningitis recurrence. The risks and benefit of CI in this peculiar clinical circumstance are discussed after reviewing the literature.

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“…In the cohort by Jonnson, post-vaccination C2 deficient patients mounted a good response to vaccination along with a ‘more efficient’ phagocytosis via increased opsonin production secondary to a possible c1q dependent C2-independent pathway [1]. This pathway may not be effectively activated in post vaccinated C2 patients who are IgG deficient because of the role IgG plays in the c1q dependent pathway.…”

Section: Discussionmentioning

confidence: 99%

Unusual presentation of a patient with complement deficiency and immunoglobulin deficiency

Manga

Stark

2014

All Asth Clin Immun

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Vaccination against encapsulated bacteria in hereditary C2 deficiency results in antibody response and opsonization due to antibody-dependent complement activation

Cited by 23 publications

References 56 publications

Primary immunodeficiency in infection-prone children in southern Sweden: occurrence, clinical characteristics and immunological findings

Primary immunodeficiency in infection-prone children in southern Sweden: occurrence, clinical characteristics and immunological findings

Delayed cochlear implantation in post-meningitic deafness and hereditary complement C2 deficiency

Unusual presentation of a patient with complement deficiency and immunoglobulin deficiency

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