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囊性纤维化的腹部表现影像学检查

Imaging the Abdominal Manifestations of Cystic Fibrosis.

作者信息

Gillespie C D, O'Reilly M K, Allen G N, McDermott S, Chan V O, Ridge C A

机构信息

Department of Medicine, Mater Misericordiae University Hospital, Dublin, Ireland.

Department of Radiology, Mater Misericordiae University Hospital, Dublin, Ireland.

出版信息

Int J Hepatol. 2017;2017:5128760. doi: 10.1155/2017/5128760. Epub 2017 Jan 29.

Abstract

Cystic fibrosis (CF) is a multisystem disease with a range of abdominal manifestations including those involving the liver, pancreas, and kidneys. Recent advances in management of the respiratory complications of the disease has led to a greater life expectancy in patients with CF. Subsequently, there is increasing focus on the impact of abdominal disease on quality of life and survival. Liver cirrhosis is the most important extrapulmonary cause of death in CF, yet significant challenges remain in the diagnosis of CF related liver disease. The capacity to predict those patients at risk of developing cirrhosis remains a significant challenge. We review representative abdominal imaging findings in patients with CF selected from the records of two academic health centres, with a view to increasing familiarity with the abdominal manifestations of the disease. We review their presentation and expected imaging findings, with a focus on the challenges facing diagnosis of the hepatic manifestations of the disease. An increased familiarity with these abdominal manifestations will facilitate timely diagnosis and management, which is paramount to further improving outcomes for patients with cystic fibrosis.

摘要

囊性纤维化(CF)是一种多系统疾病,具有一系列腹部表现,包括涉及肝脏、胰腺和肾脏的表现。该疾病呼吸并发症管理方面的最新进展已使CF患者的预期寿命延长。随后,人们越来越关注腹部疾病对生活质量和生存的影响。肝硬化是CF患者最重要的肺外死亡原因,但CF相关肝病的诊断仍面临重大挑战。预测那些有发展为肝硬化风险的患者的能力仍然是一项重大挑战。我们回顾了从两个学术健康中心的记录中选取的CF患者具有代表性的腹部影像学表现,以期增加对该疾病腹部表现的熟悉程度。我们回顾了它们的表现和预期的影像学表现,重点关注该疾病肝脏表现诊断所面临的挑战。对这些腹部表现有更多的了解将有助于及时诊断和管理,这对于进一步改善囊性纤维化患者的预后至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3f3a/5303593/370860f1d5fc/IJH2017-5128760.001.jpg

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