Biomaterial Database

Human pasteurized C1-inhibitor concentrate for the treatment of hereditary angioedema due to C1-inhibitor deficiency. 2011

Konrad Bork

Department of Dermatology, Johannes Gutenberg University, Langenbeckstr. 1, 55131 Mainz, Germany. bork@hautklinik.klinik.uni-mainz.de

Hereditary angioedema is a relatively rare genetic disorder affecting between one in 10,000 and one in 50,000 individuals worldwide. The most common clinical symptoms observed are relapsing swelling of the skin and abdominal pain attacks. However, more serious and potentially fatal laryngeal attacks can also occur. Hereditary angioedema is most frequently caused by a deficiency of C1-inhibitor. Replacement therapy with Berinert, an intravenous pasteurized C1-inhibitor concentrate derived from human plasma, is a recommended treatment for rapid resolution of acute attacks of hereditary angioedema due to C1-inhibitor deficiency. Prophylactic therapy with C1-inhibitor is also available. Future advances may improve morbidity and mortality associated with hereditary angioedema.

UI	MeSH Term	Description	Entries
D007262	Infusions, Intravenous	The long-term (minutes to hours) administration of a fluid into the vein through venipuncture, either by letting the fluid flow by gravity or by pumping it.	Drip Infusions,Intravenous Drip,Intravenous Infusions,Drip Infusion,Drip, Intravenous,Infusion, Drip,Infusion, Intravenous,Infusions, Drip,Intravenous Infusion
D003174	Complement C1 Inactivator Proteins	Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.	Complement 1 Esterase Inhibitors,Complement C1 Inactivating Proteins,Complement C1 Inhibiting Proteins,Complement C1 Inhibitor Proteins,Complement C1r Protease Inhibitor Proteins,Complement C1s Esterase Inhibitor Proteins,Complement Component 1 Inactivator Proteins
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D015746	Abdominal Pain	Sensation of discomfort, distress, or agony in the abdominal region.	Colicky Pain,Abdominal Pains,Colicky Pains,Pain, Abdominal,Pain, Colicky,Pains, Abdominal,Pains, Colicky
D050718	Complement C1 Inhibitor Protein	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.	C1 Esterase Inhibitor,C1-INH Protein,C1-Inhibitor Protein,Complement C1-Inhibitor Protein,Plasma Protease C1 Inhibitor,SERPING1,Serpin Family G Member 1,Serpin G1,C1 INH Protein,C1 Inhibitor Protein,Esterase Inhibitor, C1
D054179	Angioedemas, Hereditary	Inherited disorders that are characterized by subcutaneous and submucosal EDEMA in the upper RESPIRATORY TRACT and GASTROINTESTINAL TRACT.	Angioedema, Hereditary,Angioneurotic Edema, Hereditary,C1 Esterase Inhibitor Deficiency,C1 Inhibitor Deficiency,Hereditary Angioedema,Hereditary Angioedemas,Hereditary Angioneurotic Edema,Angioneurotic Edemas, Hereditary,Edema, Hereditary Angioneurotic,Edemas, Hereditary Angioneurotic,Hereditary Angioneurotic Edemas
D061352	Pasteurization	Treatment of food with physical methods such as heat, high pressure, radiation, or electric current to destroy organisms that cause disease or food spoilage.	Ultrapasteurization,Pasteurizations,Ultrapasteurizations