6533b837fe1ef96bd12a2991

RESEARCH PRODUCT

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

Konrad Bork

subject

medicine.medical_specialtyAbdominal painC1 inhibitor deficiencyImmunologyComplement C1 Inactivator ProteinsC1-inhibitormedicineImmunology and AllergyHumansInfusions IntravenousAngioedemabiologybusiness.industryGenetic disorderAngioedemas Hereditaryfood and beveragesmedicine.diseaseDermatologyAbdominal PainHuman plasmaImmunologyHereditary angioedemabiology.proteinBerinert PPasteurizationmedicine.symptombusinessComplement C1 Inhibitor Protein

description

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.

10.1586/eci.11.72https://pubmed.ncbi.nlm.nih.gov/22014012