研究动态
Articles below are published ahead of final publication in an issue. Please cite articles in the following format: authors, (year), title, journal, DOI.

囊性纤维化与癌症:揭示CFTR基因在癌症易感性中的复杂作用。

Cystic Fibrosis and Cancer: Unraveling the Complex Role of CFTR Gene in Cancer Susceptibility.

发表日期:2023 Aug 24
作者: Giuseppe Fabio Parisi, Maria Papale, Giulia Pecora, Novella Rotolo, Sara Manti, Giovanna Russo, Salvatore Leonardi
来源: Experimental Hematology & Oncology

摘要:

囊胞性纤维化(CF)是一种影响多个器官,特别是肺部和消化系统的遗传性疾病。多年来,医疗护理和治疗方面的进展显著延长了患有CF的个体的预期寿命。然而,随着预期寿命的延长,对于患有CF的个体患某些类型的肿瘤可能性风险的关注也在增加。本叙述性综述旨在探讨CF、预期寿命延长以及相关肿瘤风险之间的关系。我们讨论了这种风险潜在的机制,包括慢性炎症、免疫系统失调和遗传因素。此外,我们回顾了研究CF患者发生率和肿瘤类型的研究,重点关注消化道、乳房和呼吸系统恶性肿瘤。我们还探讨了CFTR调节剂治疗对癌症风险的影响。在消化道方面,CF患者患结肠癌、胰腺癌和可能的食管癌的风险升高。造成这些升高风险的潜在机制尚不完全理解,但长期炎症、肠道微生物群的改变以及遗传因素被认为发挥作用。建议对于CF患者进行定期监测和结肠镜检查,以便早期发现和管理结肠癌。了解导致CF患者癌症发展的因素对于实施适当的监测策略和改善长期预后至关重要。需要进一步的研究以阐明相关的分子机制,并开发针对CF个体的癌症风险干预手段。
Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs and digestive system. Over the years, advancements in medical care and treatments have significantly increased the life expectancy of individuals with CF. However, with this improved longevity, concerns about the potential risk of developing certain types of cancers have arisen. This narrative review aims to explore the relationship between CF, increased life expectancy, and the associated risk for cancers. We discuss the potential mechanisms underlying this risk, including chronic inflammation, immune system dysregulation, and genetic factors. Additionally, we review studies that have examined the incidence and types of cancers seen in CF patients, with a focus on gastrointestinal, breast, and respiratory malignancies. We also explore the impact of CFTR modulator therapies on cancer risk. In the gastrointestinal tract, CF patients have an elevated risk of developing colorectal cancer, pancreatic cancer, and possibly esophageal cancer. The underlying mechanisms contributing to these increased risks are not fully understood, but chronic inflammation, altered gut microbiota, and genetic factors are believed to play a role. Regular surveillance and colonoscopies are recommended for early detection and management of colorectal cancer in CF patients. Understanding the factors contributing to cancer development in CF patients is crucial for implementing appropriate surveillance strategies and improving long-term outcomes. Further research is needed to elucidate the molecular mechanisms involved and develop targeted interventions to mitigate cancer risk in individuals with CF.