一名患有纯合子家族性高胆固醇血症的5岁儿童同期活体供肝移植与非体外循环冠状动脉搭桥术：病例报告
Concurrent Living Donor Liver Transplantation and Off-Pump Coronary Artery Bypass in a Five-Year-Old Child With Homozygous Familial Hypercholesterolemia: A Case Report.
作者信息
Kazimi Mirjalal, Mahmudov Rashad, Beydullayev Kamran, Aytayev Telman, Asadov Kanan, Rustamzada Gulam, Shindiyeva Saida, Mammadova Bibixanim, Sadigov Tural, Jafarova Shahnaz, Vatansever Safa
机构信息
Department of Surgery and Organ Transplantation, Central Customs Hospital, Baku, Azerbaijan.
Department of General Surgery, Izmir University of Economics Medical Point Hospital, Izmir, Turkey.
出版信息
Transplant Proc. 2023 Apr;55(3):676-679. doi: 10.1016/j.transproceed.2023.02.024. Epub 2023 Mar 24.
BACKGROUND
Homozygous familial hypercholesterolemia (FH) is a rare disease that causes serious cardiovascular problems and may be fatal even at an early age. Because this disease variant is rather aggressive, the effect of lipid-lowering agents and lipid apheresis remains inadequate in most cases. In patients who are not responding or tolerating available treatments, liver transplantation (LT) is the last and definitive solution. In addition to LT, the need for cardiac surgery is often substantial.
CASE REPORT
This study presents the first pediatric case of FH who underwent off-pump coronary artery bypass (OPCAB) surgery concurrent with live-donor LT (LDLT). The early postoperative period was uneventful. After a 1-year follow-up period, the patient is alive and well with normal liver functions and cholesterol levels remaining within the normal range without any lipid-lowering medical therapy.
CONCLUSION
Performing simultaneous coronary bypass and LT may be safe and feasible even for pediatric patients.
背景
纯合子家族性高胆固醇血症（FH）是一种罕见疾病，可导致严重的心血管问题，甚至在早年就可能致命。由于这种疾病变体具有相当大的侵袭性，在大多数情况下，降脂药物和血脂分离术的效果仍然不足。对于那些对现有治疗无反应或不耐受的患者，肝移植（LT）是最后的决定性解决方案。除了肝移植，心脏手术的需求通常也很大。
病例报告
本研究介绍了首例接受非体外循环冠状动脉搭桥术（OPCAB）同时进行活体供肝肝移植（LDLT）的儿童FH病例。术后早期情况平稳。经过1年的随访期，患者存活且状况良好，肝功能正常，胆固醇水平保持在正常范围内，无需任何降脂药物治疗。
结论
即使对于儿科患者，同时进行冠状动脉搭桥术和肝移植可能是安全可行的。
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