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囊性纤维化的肠道并发症。

Intestinal complications of cystic fibrosis.

机构信息

Department of Pediatrics, State University of New York at Buffalo, New York, USA.

出版信息

Curr Opin Pulm Med. 2013 Nov;19(6):676-80. doi: 10.1097/MCP.0b013e3283659ef2.

Abstract

PURPOSE OF REVIEW

The article reviews advances in gastrointestinal aspects of cystic fibrosis (CF) published in the literature over the past year, and highlights new and interesting research.

RECENT FINDINGS

Animal models can be used to understand the pathophysiology of gastrointestinal complications in CF. The CF mouse is useful for studying distal intestinal obstruction, dysmotility and dysbiosis, and the CF pig model has helped us better understand meconium ileus and pancreatic and hepatobiliary secretory problems. Studies in humans help elucidate the evolution of pancreatic insufficiency, how reflux may lead to lung disease, problems with intestinal dysmotility, mechanisms leading to pancreatitis and the increased prevalence of gastrointestinal cancer. Biomarkers are shedding light on CF-related liver disease. Rectal biopsies can help in diagnosis and in studying new drugs for CF.

SUMMARY

Gastrointestinal complications of CF are likely to be seen with increasing frequency as patients with CF lead longer lives. CF animal models and modern research techniques are providing new insights into extrapulmonary complications. CF clinicians should be familiar with diagnosis and management of common gastrointestinal complications and should build bridges with specialists so that referrals can be made when needed.

摘要

目的综述

本文综述了过去一年中发表的关于囊性纤维化(CF)胃肠道方面的文献进展,重点介绍了新的有趣的研究。

最近的发现

动物模型可用于了解 CF 胃肠道并发症的病理生理学。CF 小鼠可用于研究远端肠梗阻、运动障碍和菌群失调,CF 猪模型有助于我们更好地了解胎粪性肠梗阻以及胰腺和肝胆分泌问题。对人类的研究有助于阐明胰腺功能不全的演变、反流如何导致肺部疾病、肠道运动障碍问题、导致胰腺炎的机制以及胃肠道癌症的高发率。生物标志物为 CF 相关肝病提供了新的见解。直肠活检有助于诊断和研究 CF 的新药。

总结

随着 CF 患者寿命的延长,CF 的胃肠道并发症可能会越来越常见。CF 动物模型和现代研究技术为肺外并发症提供了新的见解。CF 临床医生应该熟悉常见胃肠道并发症的诊断和治疗,并与专家建立联系,以便在需要时进行转诊。

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