Title |
Diseases of complement dysregulation—an overview
|
---|---|
Published in |
Seminars in Immunopathology, January 2018
|
DOI | 10.1007/s00281-017-0663-8 |
Pubmed ID | |
Authors |
Edwin K. S. Wong, David Kavanagh |
Abstract |
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease. Eculizumab has revolutionized the treatment of PNH and aHUS although has been less successful in C3G. With the next generation of complement therapeutic in late stage development, these archetypal complement diseases will provide the initial targets. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
United Kingdom | 1 | 13% |
India | 1 | 13% |
Unknown | 6 | 75% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 8 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 165 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Researcher | 23 | 14% |
Student > Ph. D. Student | 20 | 12% |
Student > Master | 20 | 12% |
Student > Bachelor | 17 | 10% |
Other | 15 | 9% |
Other | 27 | 16% |
Unknown | 43 | 26% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 46 | 28% |
Biochemistry, Genetics and Molecular Biology | 24 | 15% |
Pharmacology, Toxicology and Pharmaceutical Science | 15 | 9% |
Agricultural and Biological Sciences | 11 | 7% |
Immunology and Microbiology | 6 | 4% |
Other | 16 | 10% |
Unknown | 47 | 28% |