肝脏淀粉样变性一例报告及文献复习-现代医学

肝脏淀粉样变性一例报告及文献复习

单位：南京医科大学鼓楼临床医学院肝胆外科, 江苏南京 210008

分类号：R597.2

出版年·卷·期（页码）：2018·46·第二期（222-225）

摘要：

目的：探讨提高肝脏淀粉样变性的诊疗水平的策略与方法。方法：对2016年南京鼓楼医院肝胆外科收治的一例肝脏淀粉样变性患者的临床表现、影像学检查、治疗及预后恢复情况等进行回顾性分析。结果：患者出现症状后在B超定位下行肝脏穿刺活检，病理高度怀疑淀粉样变。行同种异体原位肝移植术治疗后目前患者病情平稳。术后常规抗排斥治疗同时，予优思弗及小剂量甲泼尼龙片配合治疗。结论：肝脏淀粉样变性临床表现、生化检查及影像学检查均缺乏诊断特异性，临床常被误诊，最终需要肝脏穿刺或术后病理行特殊染色确诊。部分患者保守治疗后病情可缓解，而对于终末期患者，肝移植手术是目前治疗的最佳方法。

Objective: To explore the diagnosis and treatment of hepatic amyloidosis. Methods: Clinical manifestation, imaging features, treatment and prognosis were studied within a case of hepatic amyloidosis admitted by Nanjing Drum Tower Hospital. Result: After the patient had symptoms, liver biopsy was performed under B-ultrasound guidance, and the pathology of liver biopsy highly suspected amyloidosis.The patient is currently in stable condition after allogeneic orthotopic liver transplantation.We prescribed patients with conventional oral anti-rejection drugs, as well as ursodeoxychoic acid capsules and low-dose methylprednisolone tablets. Conclusion: Hepatic amyloidosis lacks of diagnostic specificityin the aspect of clinical manifestations, biochemical tests and imaging examination, thus it is often misdiagnosed, finally needs liver puncture/postoperative pathology toconfirme. Some patients can be relieved after conservative treatment, but for end-stage patients, liver transplantation is the best treatment of the current method.

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