Cinquini Chiara, Santarelli Simonetta, Marianelli Alberto, Nisi Marco, Gabriele Mario, Barone Antonio
Department of Surgical, Medical, Molecular and of Critical Area Pathologies, Complex Operative Unit of Stomatology and Oral Surgery, University-Hospital of Pisa, University of Pisa, 56126 Pisa, Italy.
Case Rep Dent. 2022 Jan 29;2022:6602411. doi: 10.1155/2022/6602411. eCollection 2022.
Hereditary Angioedema (HAE) is a rare disease characterized by a deficiency or a reduced function of the plasma protein C1 esterase inhibitor (C1-INH), which is involved in the downregulation of several inflammatory pathways. Patients affected by HAE suffer from episodic swellings of subcutaneous or submucosal tissues. Swellings can be caused by stress or dental and surgical procedures and can be life-threatening if the airways are involved. We have reported a clinical case of a patient affected by HAE type I who underwent oral surgery procedures under a short-term prophylaxis with C1-INH plasma-derived concentrate. The patient underwent a cyst removal, multiple tooth extractions, and an excisional biopsy with a prophylaxis with C1-INH plasma-derived concentrate and was hospitalized for 36 hours after the surgery to be monitored for possible HAE attacks. During the hospitalization, the patient did not show signs of swelling nor of HAE attacks. At 14 and 28 days after the surgery, the patient presented a good surgical healing. The prophylactic intravenous infusion of C1-INH concentrate was successful in preventing acute HAE attacks after oral surgery procedures.
遗传性血管性水肿(HAE)是一种罕见疾病,其特征是血浆蛋白C1酯酶抑制剂(C1-INH)缺乏或功能降低,该抑制剂参与多种炎症途径的下调。患有HAE的患者会出现皮下或粘膜下组织的发作性肿胀。肿胀可能由压力或牙科及外科手术引起,如果累及气道则可能危及生命。我们报告了一例I型HAE患者的临床病例,该患者在短期预防性使用C1-INH血浆源性浓缩物的情况下接受了口腔外科手术。患者接受了囊肿切除、多颗牙齿拔除以及切除活检,并使用C1-INH血浆源性浓缩物进行预防,术后住院36小时以监测可能的HAE发作。住院期间,患者未出现肿胀迹象或HAE发作。术后14天和28天,患者手术愈合良好。预防性静脉输注C1-INH浓缩物成功预防了口腔外科手术后的急性HAE发作。