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Abstract：
Autosomal dominant polycystic liver disease (ADPLD) is a rare disease with variable clinical presentations, characterized by cystic enlargement of the liver. The diagnosis is made based on family history, patient\'s age, and liver phenotype and is confirmed by imaging tests. The treatment aims to reduce symptoms caused by the increased liver volume and can be performed by aspiration with sclerotherapy, fenestration, and liver resection. Although ADPLD is a rare disease, it is an important differential diagnosis of cystic diseases such as polycystic kidney disease; therefore, the aim of this article was to present the diagnostic and therapeutic approach of a case of ADPLD and conducting a literature review. This is the case of a 32-year-old male patient, who was hospitalized due to abdominal pain, hepatomegaly, lack of appetite, and weight loss. Imaging propaedeutics showed a significant increase in the liver volume due to hepatic cysts. After a multidisciplinary evaluation, given the clinical changes and the location of the hepatic cysts, fenestration was performed by laparotomy. The postoperative period was uneventful. The treatment was efficient in promoting symptomatic relief and improving the quality of life in this patient. Case reports on this disease are quite limited in the currently available literature, and there are gaps in knowledge with regard to the diagnosis and management of ADPLD. The importance of this article is that it will highlight the limitations in treatment options and allow physicians to make a more informed decision when diagnosing and treating a patient with ADPLD in the future.
摘要：
常染色体显性遗传性多囊性肝病（ADPLD）是一种罕见的疾病，临床表现各不相同，以肝脏囊性肿大为特征。诊断是根据家族史做出的，病人的年龄，和肝脏表型，并通过影像学检查证实。该治疗旨在减少由肝脏体积增加引起的症状，并且可以通过硬化疗法进行抽吸，开窗术,和肝切除术.虽然ADPLD是一种罕见的疾病，它是多囊肾等囊性疾病的重要鉴别诊断;因此,本文的目的是介绍一例ADPLD的诊断和治疗方法,并进行文献综述.这是一个32岁的男性患者的案例，他因腹痛住院，肝肿大,缺乏食欲,和减肥。成像方案显示，由于肝囊肿，肝体积显着增加。经过多学科评估，鉴于临床变化和肝囊肿的位置，开窗术通过剖腹手术进行.术后时间顺利。该治疗可有效促进该患者的症状缓解并改善其生活质量。在目前可用的文献中，关于这种疾病的病例报告相当有限，在ADPLD的诊断和管理方面存在知识空白。本文的重要性在于，它将强调治疗方案的局限性，并允许医生在将来诊断和治疗ADPLD患者时做出更明智的决定。
