Investigation for complement deficiency following meningococcal disease

Hoare, Simon; Elshazali, Osama Hafiz; Clark, Julia; Fay, André P.; Cant, Andrew J.

doi:10.1136/adc.86.3.215

Cited by 38 publications

(20 citation statements)

References 8 publications

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“…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.…”

Section: Frequency Of Hereditary Complement Deficiencies Among Patienmentioning

confidence: 99%

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

2010

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SUMMARY The complement system comprises several fluid-phase and membrane-associated proteins. Under physiological conditions, activation of the fluid-phase components of complement is maintained under tight control and complement activation occurs primarily on surfaces recognized as “nonself” in an attempt to minimize damage to bystander host cells. Membrane complement components act to limit complement activation on host cells or to facilitate uptake of antigens or microbes “tagged” with complement fragments. While this review focuses on the role of complement in infectious diseases, work over the past couple of decades has defined several important functions of complement distinct from that of combating infections. Activation of complement in the fluid phase can occur through the classical, lectin, or alternative pathway. Deficiencies of components of the classical pathway lead to the development of autoimmune disorders and predispose individuals to recurrent respiratory infections and infections caused by encapsulated organisms, including Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. While no individual with complete mannan-binding lectin (MBL) deficiency has been identified, low MBL levels have been linked to predisposition to, or severity of, several diseases. It appears that MBL may play an important role in children, who have a relatively immature adaptive immune response. C3 is the point at which all complement pathways converge, and complete deficiency of C3 invariably leads to severe infections, including those caused by meningococci and pneumococci. Deficiencies of the alternative and terminal complement pathways result in an almost exclusive predisposition to invasive meningococcal disease. The spleen plays an important role in antigen processing and the production of antibodies. Splenic macrophages are critical in clearing opsonized encapsulated bacteria (such as pneumococci, meningococci, and Escherichia coli) and intraerythrocytic parasites such as those causing malaria and babesiosis, which explains the fulminant nature of these infections in persons with anatomic or functional asplenia. Paramount to the management of patients with complement deficiencies and asplenia is educating patients about their predisposition to infection and the importance of preventive immunizations and seeking prompt medical attention.

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Section: Frequency Of Hereditary Complement Deficiencies Among Patienmentioning

confidence: 99%

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

2010

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“…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].…”

Section: Discussionmentioning

confidence: 78%

Meningococcal disease associated with an acute post-streptococcal complement deficiency

et al. 2007

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Chronic deficiencies in the complement pathway proteins are associated with an increased risk of meningococcal disease. Such deficiencies are caused by primary congenital immunodeficiency of a complement protein, properdin or mannose binding lectin, or are secondary to consumption of complement by systemic lupus erythematosus (SLE) or membranoproliferative glomerulonephritis (MPGN). Whatever the cause, the complement deficiency is always chronic. Here we report a case of meningococcal disease (MCD) in a child with a transient complement deficiency (CD), caused by post-streptococcal glomerulonephritis (PSGN).

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“…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.…”

Section: Warning Signs For Microbiologists/infectious Diseases Physicmentioning

confidence: 90%

The 10 warning signs

O’Sullivan

Cant

2012

Current Opinion in Allergy &Amp; Clinical Immunology

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Investigation for complement deficiency following meningococcal disease

Cited by 38 publications

References 8 publications

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

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

Meningococcal disease associated with an acute post-streptococcal complement deficiency

The 10 warning signs

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