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T1  - European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) Complement Guideline : Deficiencies, Diagnosis, and Management 
SN  -  /  
UR  - http://hdl.handle.net/10138/316915 
T3  -  
A1  - Brodszki, Nicholas; Frazer-Abel, Ashley; Grumach, Anete S.; Kirschfink, Michael; Litzman, Jiri; Perez, Elena; Seppänen, Mikko R. J.; Sullivan, Kathleen E.; Jolles, Stephen 
A2  -  
PB  -  
Y1  - 2020 
LA  - eng 
AB  - This guideline aims to describe the complement system and the functions of the constituent pathways, with particular focus on primary immunodeficiencies (PIDs) and their diagnosis and management. The complement system is a crucial part of the innate immune system, with multiple membrane-bound and soluble components. There are three distinct enzymatic cascade pathways within the complement system, the classical, alternative and lectin pathways, which converge with the cleavage of central C3. Comp... 
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KW  - Complement; complement deficiencies; classical pathway; alternative pathway; mannan-binding lectin; MANNOSE-BINDING LECTIN; STEM-CELL TRANSPLANTATION; HEMOLYTIC-UREMIC SYNDROME; HUMAN C1Q DEFICIENCY; GENE POLYMORPHISM; HEREDITARY ANGIOEDEMA; PROPERDIN DEFICIENCY; MACULAR DEGENERATION; FACTOR-H; CLINICAL PRESENTATION; 3111 Biomedicine 
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