The life expectancy of cystic fibrosis (CF) patients has greatly increased over the past decade, and researchers and clinicians must now navigate complex disease manifestations that were not a concern prior to the development of modern therapies. Explosive growth in the number of CF animal models has also occurred over this time span, clarifying CF disease pathophysiology and creating opportunities to understand more complex disease processes associated with an aging CF population. This review focuses on the CF-associated pathologies of the gastrointestinal system and how animal models have increased our understanding of this complex multisystemic disease. Although CF is primarily recognized as a pulmonary disease, gastrointestinal pathology occurs very commonly and can affect the quality of life for these patients. Furthermore, we discuss how next-generation genetic engineering of larger animal models will impact the field's understanding of CF disease pathophysiology and the development of novel therapeutic strategies.

中文翻译：

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动物模型及其在了解囊性纤维化相关胃肠道病变的病理生理学中的作用。

在过去十年中，囊性纤维化 (CF) 患者的预期寿命大大增加，研究人员和临床医生现在必须应对复杂的疾病表现，这些表现在现代疗法开发之前并不值得关注。在此期间，CF 动物模型的数量也出现了爆炸性增长，阐明了 CF 疾病的病理生理学，并为了解与老龄化 CF 人口相关的更复杂的疾病过程创造了机会。这篇综述的重点是胃肠系统的 CF 相关病理学以及动物模型如何增加我们对这种复杂的多系统疾病的理解。尽管 CF 主要被认为是一种肺部疾病，但胃肠道病变非常常见，并且会影响这些患者的生活质量。此外，