Department of Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan.
Ann Thorac Surg. 2011 Sep;92(3):1104-5. doi: 10.1016/j.athoracsur.2011.03.051.
Scheie's syndrome is a rare hereditary disorder of proteoglycan-degrading enzymes. Deposition of mucopolysaccharide can cause valvular and other tissue abnormalities. Few studies have reported surgical results for Scheie's syndrome, and the perioperative course is complicated. This is a report of a 35-year-old woman with Scheie's syndrome who underwent double-valve replacement with intensive perioperative management.
Scheie 综合征是一种罕见的蛋白聚糖降解酶遗传性疾病。黏多糖的沉积可导致瓣膜和其他组织异常。很少有研究报道 Scheie 综合征的手术结果，且围手术期过程复杂。本文报告了 1 例 35 岁女性 Scheie 综合征患者，采用强化围手术期管理行双瓣膜置换术。
