Investigation for complement deficiency following meningococcal disease

Abstract

Background and aims: The incidence of complement abnormalities in the UK is not known. It is suggested in at least three major paediatric textbooks to test for abnormalities of the complement system following meningococcal disease (MCD). Methods: Over a four year period, surviving children with a diagnosis of MCD had complement activity assessed. A total of 297 children, aged 2 months to 16 years were screened. Results: All children except one had disease caused by B or C serogroups. One child, with group B meningococcal septicaemia (complicated by disseminated intravascular coagulation and who required ventilation and inotropic support) was complement deficient. C2 deficiency was subsequently diagnosed. She had other major pointers towards an immunological abnormality prior to her MCD.

Infections of People with Complement Deficiencies and Patients Who Have Undergone Splenectomy

“…A relatively high frequency of C6 deficiency has been detected in the western Cape population of South Africa (330,358). The association of complement deficiencies with invasive neisserial infections prompted several studies to estimate the frequency of complement deficiencies among patients with their first episode of systemic neisserial infection (24,80,94,109,111,114,134,192,247,313,321,351,363,393). Figueroa and Densen (128) plotted the incidence of complement deficiency detected in patients with meningococcal disease versus the incidence of meningococcal disease in the general population and showed that the rate of complement deficiencies detected dropped in areas where the incidence of disease was high.…”

Meningococcal disease associated with an acute post-streptococcal complement deficiency

“…The high number of cases with CD seen in the first series may have been influenced by the low incidence of MCD in their population, as a greater proportion of children with CD would be expected in such areas [8]. In contrast, results from a country of high MCD incidence only revealed one case of complement deficiency in 297 cases of MCD [10].…”

The 10 warning signs

“…Routine screening for complement deficiency in children with one episode of meningococcal disease is not supported by existing data from northern European children if caused by the serotypes most prevalent in that population; only one of 296 children investigated in the United Kingdom with group B or C infection was found to have a complement deficiency and that child had a history of recurrent invasive infection with encapsulated organisms [41]. However, if a less common Neisseria meningitidis serotype (such as W135 or Y in the United Kingdom) is isolated or there is recurrent infection or a family history of meningococcal disease, further investigation is warranted.…”