Suzuki T, Tsuchiyama Y, Kawaguchi Y, Sougawa T, Nakamura H, Kinoshita T
Masui. 1989 Nov;38(11):1518-21.
Emergency anesthesia was carried out for a patient with Ehlers-Danlos syndrome (type IV) and factor XIII deficiency. The operation was a ligation of the bleeding right subclavian artery. One month later, a transverse colectomy and splenectomy was performed for a necrotic perforation of the colon, but the wound did not heal completely. The patient died of another perforation of the intestine two months later. Anesthetic management of a patient with Ehlers-Danlos syndrome and factor XIII deficiency was reviewed. Particular problems concerning a genetic disease were also discussed.
为一名患有埃勒斯-当洛综合征（IV型）和XIII因子缺乏症的患者实施了急诊麻醉。手术为结扎出血的右锁骨下动脉。一个月后，因结肠坏死穿孔进行了横结肠切除术和脾切除术，但伤口未完全愈合。患者在两个月后因肠道再次穿孔死亡。回顾了埃勒斯-当洛综合征和XIII因子缺乏症患者的麻醉管理。还讨论了与一种遗传性疾病相关的特殊问题。
