Usefulness of C1 esterase inhibitor protein concentrate in the management of hereditary angioedema of oropharyngeal tissue
Nigerian Journal of Clinical Practice, cilt.21, sa.4, ss.531-533, 2018 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 21 Sayı: 4
- Basım Tarihi: 2018
- Doi Numarası: 10.4103/njcp.njcp_177_17
- Dergi Adı: Nigerian Journal of Clinical Practice
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Sayfa Sayıları: ss.531-533
- Anahtar Kelimeler: C1 esterase inhibitor protein, hereditary angioedema, laryngeal edema, oropharyngeal swelling
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Hereditary angioedema is an autosomal-dominant disorder caused by mutation of the gene encoding the C1 esterase inhibitor (C1-INH). It manifests as painless, nonpruritic, nonpitting episodic swelling of the subcutaneous tissues, gastrointestinal, and upper respiratory tracts in the absence of urticaria. An attack typically takes 24 h to peak and resolves over 48-72 h. The most serious manifestation is a laryngeal attack associated with upper airway swelling. The aim of this case report is to describe the lifesaving use of a novel C1-INH protein concentrate in a patient with mild-to-moderate dyspnea caused by swelling of the upper airway (larynx) and tongue.