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- W4293157546 abstract "The complement system is comprised of both soluble proteins and membrane-bound regulators that evolved to provide host defense, primarily to bacterial pathogens. Defects in these proteins can cause significant human disease. The major phenotypes seen in complement deficiencies are systemic lupus erythematosus, infection, atypical hemolytic uremic syndrome, and angioedema. The diagnosis and management must be individualized according to the clinical picture, and in many cases genetic testing is required for a clear diagnosis. Deficiencies of the classical pathway components are associated with infection and systemic lupus erythematosus because these components function to clear apoptotic cells, participate in B cell tolerance, and cleave C3, a major opsonin. Deficiencies of the terminal components are associated with susceptibility to neisserial infections. These components collectively are responsible for bacteriolysis of Gram-negative bacteria. Defects in the regulatory components are associated either with infection if they lead to a secondary deficiency of C3 or atypical hemolytic uremic syndrome if consumption of components is unregulated. Activation of complement on the endothelium drives this disease process. Treatment, such as the diagnostic strategy must be individualized to each patient depending on their disease manifestations." @default.
- W4293157546 created "2022-08-27" @default.
- W4293157546 creator A5005713194 @default.
- W4293157546 date "2023-01-01" @default.
- W4293157546 modified "2023-09-27" @default.
- W4293157546 title "Inherited Complement Deficiencies" @default.
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