P255 Meningococcal vaccination in children with terminal complement complex deficiency

Background and aimsPatients with persistent deficiencies in the complement pathway have an increased risk for meningococcal infection and can experience recurrent disease. In this group we can include genetic deficiencies, functional or anatomic asplenia and complement blocking therapies.MethodsTwo...

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Published in:Archives of disease in childhood 2017-06, Vol.102 (Suppl 2), p.A133-A133
Main Authors: Catalin, Lungu Adrian, Andrei, Capitanescu, Alexandra, Niculae, Iulia, Constantinescu, Ileana, Negru, Camelia, Pantoc, Elena, Marin Anca, Elena, Baducu Diana, Ana-Maria, Viziniuc, Cristina, Stoica
Format: Article
Language:English
Subjects:
Asplenia
Complement factor H
Complement system
Genetic screening
Haplotypes
Hemolytic uremic syndrome
Immunization
Immunoglobulin G
Neisseria
Patients
Pediatrics
Prophylaxis
Recurrent infection
Serotypes
Vaccines
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Summary:Background and aimsPatients with persistent deficiencies in the complement pathway have an increased risk for meningococcal infection and can experience recurrent disease. In this group we can include genetic deficiencies, functional or anatomic asplenia and complement blocking therapies.MethodsTwo patients with atypical hemolytic uremic syndrome confirmed by genetic testing, one with Complement Factor H mutation, Homozygous Risk Haplotype and Hheterozygous Risk Haplotype in MCP, the other one with Anti-CFH Antibodies and Complement Factor H Related Protein 1 and 3 Homozygous Deletions, under long time complement blocking therapy with Eculizumab were vaccinated for Neisseria Meningitidis. We used the two commercially available vaccines in Europe for serotypes A, C, W135, Y and for serotype B.ResultsVaccine reaction was tested after one year by measuring the IgG antibody for Neisseria Meningitidis serotype A, C, W135, Y and B.In both patients we obtained good vaccine reaction for each Neisseria Meningitidis serotypes with IgG level over the upper laboratory limits.ConclusionsIn patients with terminal complement complex deficiency, congenital or acquired, Neisseria Meningitidis vaccination is mandatory.In Europe, Neisseria Meningitidis serotype B is involved in more than 75% of infections, so vaccination for this strain is important.Because Complement System is involved in the defense against these bacteria, even if we obtain a good vaccine reaction, we can not be sure if this assures adequate disease protection.Long time antibiotic prophylaxis and close monitoring of these patients may be required.
ISSN:0003-9888
1468-2044
DOI:10.1136/archdischild-2017-313273.343