Gugilă I, Ruxanda Anca, Vasile L, Iordache Violeta
Clinica III Chirurgie, Spitalul Clinic de Urgenţă Craiova, Bd. Mareşal Ion Antonescu, nr. 60, 1100, Craiova.
Chirurgia (Bucur). 2003 Sep-Oct;98(5):437-41.
The authors are presenting one case of Osler's hereditary angioneurotic oedema, rare genetic disease with dominant autosomal transmission linked to the 11-th chromosome, with clinical aspects resembling to those of surgical acute abdomen, with difficult diagnostic problems. The treatment consist in: fresh plasma administration, antihistaminic drugs and anabolic steroids. The simple laparotomy under general anaesthesia by orotraheal intubation being very dangerous. The patients with Osler's hereditary angioneurotic oedema must be followed-up by the allergology services and educated regarding the disease and it's risks to avoid diagnostic errors with following negative consequences.
作者报告了一例奥斯勒遗传性血管神经性水肿病例,这是一种罕见的常染色体显性遗传病,与第11号染色体相关,其临床症状与外科急腹症相似,诊断存在困难。治疗方法包括:输注新鲜血浆、使用抗组胺药物和合成代谢类固醇。在全身麻醉下行经口气管插管的单纯剖腹手术非常危险。患有奥斯勒遗传性血管神经性水肿的患者必须由过敏科进行随访,并接受有关该疾病及其风险的教育,以避免出现诊断错误及随之而来的不良后果。