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Hereditary angioedema: approaches to diagnosis and treatment, analysis of a clinical family case
Hereditary angioedema belongs to the group of rare, orphan, genetically determined defects that represent a significant medical and social problem due to the pronounced impact on the quality of life and potential mortality, as well as the emerging ...
O. V. Skorohodkina +3 more
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Expression of platelet C1 inhibitor [PDF]
Abstract Human platelets contain a pool of C1 inhibitor (C1 INH) distinct from that in plasma. Twelve normal platelet samples washed by centrifugation had a mean platelet C1 INH antigen level of 19.3 +/- 2.8 ng (mean +/- SEM) per 10(8) platelets. These values contrast with the mean +/- SEM platelet C1 INH antigen level of 6.1 +/- 0.9 per
A H, Schmaier +4 more
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Optimum Use of Acute Treatments for Hereditary Angioedema: Evidence-Based Expert Consensus
Acute treatment of hereditary angioedema due to C1 inhibitor deficiency has become available in the last 10 years and has greatly improved patients’ quality of life.
Hilary Longhurst
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Biological activities of C1 inhibitor [PDF]
Broadly speaking, C1 inhibitor plays important roles in the regulation of vascular permeability and in the suppression of inflammation. Vascular permeability control is exerted largely through inhibition of two of the proteases involved in the generation of bradykinin, factor XIIa and plasma kallikrein (the plasma kallikrein-kinin system).
Alvin E, Davis +2 more
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2010 international consensus algorithm for the diagnosis, therapy and management of hereditary angioedema [PDF]
Background We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art ...
S. Waserman +251 more
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C1 inhibitor of serine proteases (C1-INH) performs a regulatory function in the complement system and vascular permeability. Deficiency of C1-INH leads to various forms of angioedema, including hereditary angioedema (HAE).
N. P. Gorbunov +12 more
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Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx are affected by attacks of swelling.
Coleman Suzanne +2 more
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The authors wish to make corrections to their article: Nicola S, Rolla G, Brussino L. Breakthroughs in hereditary angioedema management: a systematic review of approved drugs and those under research. Drugs in Context 2019; 8: 212605. DOI: 10.7573/dic.
Stefania Nicola +2 more
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Background. Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is caused by dysfunctional C1-INH protein due to mutations in the SERPING1 gene encoding C1-INH.
Ezgi Topyıldız +4 more
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C1-Inhibitor and Transplantation
Excessive activation of the protein cascade systems has been associated with post-transplantation inflammatory disorders. There is increasing evidence that complement not only significantly contributes to ischemia/reperfusion injury upon cold storage of the organ but also, although to a different degree, to allograft rejection. Complement activation is
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