血栓弹力图在伴有血小板功能障碍的雅各布森综合征心脏手术中的应用：一例报告
Utility of thromboelastogram in cardiac surgery in Jacobsen syndrome associated with platelet dysfunction: a case report.
作者信息
Takeda Chikashi, Hirotsu Akiko, Yasuhara Gento, Mizuno Akito, Tatsumi Kenichiro, Kawamoto Shuji
机构信息
Department of Anesthesia, Kyoto University Hospital, Kyoto, 606-8507, Japan.
出版信息
JA Clin Rep. 2022 Aug 22;8(1):67. doi: 10.1186/s40981-022-00557-w.
BACKGROUND
Jacobsen syndrome is a rare genetic disorder with multiple congenital anomalies and platelet abnormalities caused by chromosome 11 deletion.
CASE PRESENTATION
A 7-month-old boy with thrombocytopenia underwent ventricular septal defect closure. At the beginning of surgery, the platelet count was 168 × 10/μL, and heparinized kaolin with heparinase reaction time (HKH-R), which represents clot formation time, was prolonged at 30.4 min. Platelet transfusion was continued, and at the end of surgery, the platelet count and HKH-R values improved to 215 × 10/μL and 15 min, respectively.
CONCLUSIONS
As anesthetic management of patients with abnormal platelet function, the viscoelasticity test might be useful in evaluating hemostatic capacity.
背景
雅各布森综合征是一种罕见的遗传性疾病，由11号染色体缺失导致多种先天性异常和血小板异常。
病例报告
一名7个月大的血小板减少男孩接受了室间隔缺损修补术。手术开始时，血小板计数为168×10/μL，代表凝血形成时间的肝素化高岭土与肝素酶反应时间（HKH-R）延长至30.4分钟。持续进行血小板输注，手术结束时，血小板计数和HKH-R值分别改善至215×10/μL和15分钟。
结论
作为血小板功能异常患者的麻醉管理，粘弹性试验可能有助于评估止血能力。
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