Successful long-term prophylaxis with human plasma-derived C1 inhibitor in planning and carrying out pregnancy

• Published in: Allergology International Vol. 67; no. 1; pp. 144 - 146
• Main Authors: Susana Calaforra-Mendeza, Ethel Ibanez Echevarriaa, Carolina Perales Chordaa, Maria Veronica Pacheco-Coronelc, Agustin Fernandez Llopezb, Dolores Hernandez Fernandez de Rojasa
• Format: Journal Article
• Language: Japanese
• Published: JAPANESE SOCIETY OF ALLERGOLOGY 01.01.2018
• ISSN: 1323-8930; 1440-1592

Dear Editor, Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disorder characterized by recurrent attacks of edema in different locations, which may be peripheral or affect internal tissues, as the gastrointestinal tract causing abdominal pain or the upper airways, producing life-threatening asphyxia. The physiopathology of the disease involves several vasoactive peptides, such as bradykinin, which increase endothelial permeability and vascular leakage. The C1 inhibitor (C1-INH) quantitative or qualitative deficiency is the characteristic biomarker of the disease. The clinical manifestations reduce the physical and social functioning with a significant impact in the quality of life of patients. Frequent attack triggers are stress, trauma and infections. Direct and indirect costs of the disease have been estimated on 42 000 US$ a year per patient, but when attack severity and frequency are considered, costs rise up to 92 000 US$. Therapy is addressed by treating or preventing acute attacks and the regimen should always be individualized.