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目的:总结糖原贮积症Ⅱ型供者肝、肾移植的短期临床效果，探讨其可行性。方法:回顾性分析2019年2月广州医科大学第三附属医院及南京鼓楼医院糖原贮积症Ⅱ型供者肝、肾移植3例术后的转氨酶、胆红素、血肌酐水平及B型超声检查的器官功能情况。结果:目前3例受者移植器官功能良好，例1肝移植受者术后15个月复查天冬氨酸转氨酶(AST)35 U/L、谷氨酸转氨酶(ALT)12.6 U/L、总胆红素28.3 μmol/L、直接胆红素(DBIL)4.2 μmol/L，B型超声检查提示肝静脉及门静脉、肝动脉血流通畅，移植肝实质回声均匀。例2为左肾受者术后14个月复查血肌酐水平为95 μmol/L，例3为右肾受者术后14个月复查血肌酐水平为78 μmol/L，肾脏彩色超声多普勒检查提示移植肾血流丰富，动脉阻力指数正常，移植器官近期预后佳。结论:可考虑糖原贮积症Ⅱ型供者捐献器官行肝、肾移植；术前应进行实验室检查及病理组织学评估；术后加强随访，警惕移植器官糖原贮积症发生、进展。“,”Objective:As a rare autosomal recessive genetic disease, glycogen accumulation type may involve all body organs and tissues. This report was intended to summarize the short-term clinical efficacies of liver and kidney transplantation from donors with Pompe disease and examine its feasibility.Methods:The serum levels of transaminase, bilirubin and creatinine and organ functions under B-mode ultrasound were retrospectively analyzed for 3 recipients of organs from donor with Pompe disease at Third Affiliated Hospital, Guangzhou Medical University and Nanjing Drum Tower Hospital in February 2019.Results:Currently organ functions of three recipients were stable. At 15 months post-operation, one liver transplant recipient were reexamined with the results of aspartate aminotransferase (AST) 35 U/L, glutamate transaminase (ALT) 12.6 U/L, total bilirubin (TBIL) 28.3 μmol/L and direct bilirubin (DBIL) 4.2 μmol/L. B-ultrasonography revealed that blood flows were unobstructed for three hepatic veins, portal vein and hepatic artery and there was non-homogeneous echo of transplanted liver parenchyma. The serum creatinine level was 95 μmol/L in one left kidney recipient and 78 μmol/L in another right kidney recipient at 14 months post-operation. Ultrasound indicated that blood flow of both transplanted kidneys was rich with a normal arterial resistance index.Conclusions:The prognosis of transplanted organs is excellent in the near future. And liver and kidney transplantation from donors is indicated for Pompe disease. Preoperative examinations and pathological evaluations should be performed. And postoperative follow-ups should be strengthened for watching out for the occurrence and progression of Pompe disease.