Hereditary angioedema
Article Abstract:
C1 inhibitor concentrate appears to be effective in treating acute attacks of hereditary angioedema. Hereditary angioedema affects 1 in 10,000 to 1 in 50,000 people and can cause life-threatening symptoms. It is caused by a defect or deficiency of the inhibitor of the C1 protein, which regulates the complement system. Acute episodes are characterized by swelling of the skin and internal organs, which can cause asphyxia if the larynx is affected.
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1996
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The inflammasome: A linebacker of innate defense
Article Abstract:
The mutations in the cold-induced autoinflammatory syndrome 1 (CIAS1) gene, which encodes cryopyrin, are found to cause two hereditary periodic-fever syndromes, the Muckle-Wells syndrome and the familial cold autoinflammatory syndrome. The cryopyrin inflammasome can be modified to protect against diverse microorganisms, increase efficiency of vaccines and ease inflammatory arthritis characteristic of gout and pseudogout.
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 2006
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