Abstract
Although the complement system was first described at the turn of the twentieth century, it was not until 1960 that the first patient with a genetically determined complement deficiency was identified. Since then, deficiencies have been described for nearly all of the components of the complement system. Individuals with genetically determined complement deficiencies have a variety of clinical presentations. Most patients present with an increased susceptibility to infections, others with a variety of rheumatic diseases, still others with angioedema, and in rare instances, patients may even be asymptomatic. The elucidation of the pathophysiological basis for the different clinical presentations of complement-deficient individuals has contributed to a better understanding of the physiological role of complement in normal individuals.
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Sullivan, K. et al. (2012). Complement Deficiencies. In: Aghamohammadi, A., Rezaei, N. (eds) Clinical Cases in Primary Immunodeficiency Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-31785-9_8
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DOI: https://doi.org/10.1007/978-3-642-31785-9_8
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