肝淀粉样变性的临床特点及随访

Hepatic amyloidosis: Clinical characteristics and follow-up

目的:通过对中国人民解放军成都军区总医院确诊为肝淀粉样变性患者临床特点、治疗及随访资料总结分析,以提高对本病的了解,从而减少误诊率.方法:回顾性分析中国人民解放军成都军区总医院收治的4例肝淀粉样变性患者临床、实验室检查、肝脏穿刺活检病理学、治疗及随访资料.结果:4例患者均有肝肿大,碱性磷酸酶、γ-谷氨酰基转移酶明显升高,部分患者血清蛋白降低,蛋白尿及高脂血症.肝脏穿刺活检染色示肝细胞及网状支架间见大量粉红色淀粉样物质沉积.经口服激素及对症治疗后均出院,随访2例患者因肝功衰竭、1例因肾功能衰竭死亡.结论:肝淀粉样变性为临床罕见疾病,需经肝穿刺活检可确诊.患者经对阵治疗后病情好转,激素药物可暂时控制病情,但患者预后不良.

AIM： To summarize the clinical features and outcomes of patients with hepatic amyloidosis to improve its diagnosis and treatment and decrease the misdiagnosis rate.METHODS： A total of 4 patients who were diagnosed with hepatic amyloidosis at General Hospital of Chengdu Military Region were retrospectively analyzed in terms of clinical features, laboratory tests, liver biopsy, treatment and follow-up data. RESULTS： All the 4 patients had hepatomegaly, increased ALP and γ-glutamyl transferase, and some patients had decreased serum albumin, proteinuria and hyperlipidemia. The liver biopsy pathology showed significant amyloid deposition between hepatocytes and mesh scaffolds. After oral steroids and symptomatic treatment, all patients had improved symptoms and were discharged. During follow-up, two patients died because of liver function failure, and 1 patient died due to kidney function failure. CONCLUSION： Hepatic amyloidosis is a rare disease, which can be diagnosed by liver biopsy pathology. Steroid hormone drugs can temporarily control the disease, although the prognosis is still poor.