Complement Deficiencies

ADLI ALI*, FLORENCE BAKON**
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Management of Complement Deficiencies
Causative therapies are currently unavailable and often unnecessary as standard treatments of complement deficiencies. Replacement of the respective complement components, either recombinant or plasma products, would have too many disadvantages: most complement factors have a short half-life and would require frequent intravenous administration which would be disproportionally inconvenient for the patients. Moreover, they would be costly, possibly immunogenic and plasma products would come with a theoretical risk of transmitting infectious agents. Even in case of acute infection, replacement therapies are redundant since the patients usually responds to treatment with antibiotics.
Resistance to infections in complement deficient patients can be increased most efficiently by active immunisation. Particularly vaccination against encapsulated bacteria is recommended (Jonsson G et al). These include Heamophilus influenza type b, Streptococcus pneumoniae and the main Neisseria meningitides serotypes A, C, W135, Y and possibly B with the introduction of the new serotype B vaccine. Subsequently, the antibody titers should be controlled to ensure that they are protective. More severe cases with recurrent or persistent serious infections might require prophylactic antibiotic therapy (Kuruvilla M et al)

The role of complement in the immune system, has expanded dramatically. It is a well-characterized and an evolving component of host defense, impairment of which leads to susceptibility to infection. Complement represents a cornerstone of the innate defense against infection and provides a vital first-line barrier by activation of proteolytic cascades which leads to the identification and persecution of the surface identified as foreign and allows complement to contain, control, and finally clear invading microorganisms. On top of these important contributions to innate immunity, complement plays a vital role in shaping adaptive immune responses, functionally integrating it into the ability of the host to combat invasion from a wide range of pathogens. The wide range of clinical manifestations of complement de?ciencies reflects the complexity of the complement system. As more individuals are identi?ed with speci?c complement de?ciencies, our understanding of how the complement system functions in innate and adaptive immunity will evolve. In conclusion, complement is a multifaceted and robust effector, which bridges the innate and adaptive immune systems. It is vital to host defense, and the extent of its influence is becoming increasingly appreciated as additional information regarding the far-reaching effects of its activation is uncovered. Further study should produce significant knowledge in our understanding of host defense as an integrated process and the roles complement plays in bridging innate and adaptive immunity.


References
Complement Deficiencies Complement Deficiencies 02/19/2016
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