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Hereditary Angioedema: An Updated Experience with Patients with Angioedema in Puerto Rico.

07:00 EST 1st December 2019 | BioPortfolio

Summary of "Hereditary Angioedema: An Updated Experience with Patients with Angioedema in Puerto Rico."

This was a study of patients with hereditary angioedema (HAE) and their responses to new therapies, measured in terms of HAE attack rates, the number of hospitalizations and emergency room (ER) visits, and the impact of HAE on their quality of life (QOL).

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Journal Details

This article was published in the following journal.

Name: Puerto Rico health sciences journal
ISSN: 2373-6011
Pages: 247-253

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Medical and Biotech [MESH] Definitions

Forms of hereditary angioedema that occur due to mutations in the gene for COMPLEMENT C1 INHIBITOR PROTEIN. Type I hereditary angioedema is associated with reduced serum levels of complement C1 inhibitor protein. Type II hereditary angioedema is associated with the production of a non-functional complement C1 inhibitor protein.

A form of hereditary angioedema that occurs in women and is precipitated or worsened by high ESTROGEN levels. It is associated with mutations in the gene for FACTOR XII that result in its increased activity.

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An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.

Stable blood coagulation factor activated by contact with the subendothelial surface of an injured vessel. Along with prekallikrein, it serves as the contact factor that initiates the intrinsic pathway of blood coagulation. Kallikrein activates factor XII to XIIa. Deficiency of factor XII, also called the Hageman trait, leads to increased incidence of thromboembolic disease. Mutations in the gene for factor XII that appear to increase factor XII amidolytic activity are associated with HEREDITARY ANGIOEDEMA TYPE III.

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