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多囊肝病

Polycystic liver disease.

作者信息

Leão Rodrigo Nazário, Salustio Raquel, Ribeiro José Vaz

机构信息

Department of Internal Medicine, Centro Hospitalar Lisboa Central, EPE, Lisboa, Portugal.

出版信息

BMJ Case Rep. 2014 Jan 17;2014:bcr2013202003. doi: 10.1136/bcr-2013-202003.

Abstract

A widespread use of ultrasound (US) examination is contributing to an increase in the diagnosis of renal and hepatic cysts. However, the vast majority of these lesions are benign with an indolent course during the patient's lifespan. Adult polycystic kidney disease (APKD) is one of the most common diagnosed entities. APKD is a genetic disease defined by the presence of multiple kidney cysts, occasionally accompanied by hepatic cysts. The presence of hepatic cysts sparing kidneys is very rare and thereby must be assumed as a different clinical entity. This article describes a case of an exuberant hepatomegaly due to the presence of isolated multiple hepatic cysts without renal involvement.

摘要

超声(US)检查的广泛应用促使肾囊肿和肝囊肿的诊断率上升。然而,这些病变绝大多数是良性的,在患者的一生中进展缓慢。成人多囊肾疾病(APKD)是最常见的诊断疾病之一。APKD是一种遗传性疾病,其特征是存在多个肾囊肿,偶尔伴有肝囊肿。单纯出现肝囊肿而无肾囊肿的情况非常罕见,因此必须将其视为一种不同的临床实体。本文描述了一例因孤立性多发性肝囊肿而无肾受累导致肝脏肿大的病例。

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Polycystic liver disease.多囊肝病
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引用本文的文献

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Hepatic cysts: a survival guide.肝囊肿:生存指南。
Radiol Bras. 2025 Apr 25;58:e20240101en. doi: 10.1590/0100-3984.2024.0101-en. eCollection 2025 Jan-Dec.

本文引用的文献

2
Systematic review: the pathophysiology and management of polycystic liver disease.系统综述:多囊肝疾病的病理生理学和治疗管理。
Aliment Pharmacol Ther. 2011 Oct;34(7):702-13. doi: 10.1111/j.1365-2036.2011.04783.x. Epub 2011 Jul 26.
5
Isolated polycystic liver disease.孤立性多囊肝病。
Adv Chronic Kidney Dis. 2010 Mar;17(2):181-9. doi: 10.1053/j.ackd.2009.12.005.
6
Carbohydrate antigen 19-9 is extremely elevated in polycystic liver disease.碳水化合物抗原19-9在多囊肝病中极度升高。
Liver Int. 2009 Oct;29(9):1389-95. doi: 10.1111/j.1478-3231.2009.02055.x. Epub 2009 Jun 3.
10
Volume progression in polycystic kidney disease.多囊肾病中的体积进展
N Engl J Med. 2006 May 18;354(20):2122-30. doi: 10.1056/NEJMoa054341.

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