Department of Molecular Biology, University of Duisburg-Essen, Hufelandstrasse 55, 45122 Essen, Germany.
Expert Rev Respir Med. 2011 Aug;5(4):527-35. doi: 10.1586/ers.11.36.
Cystic fibrosis is characterized by severe intestinal and pulmonary symptoms, but also changes in the liver, pancreas and reproductive tract. Since gastrointestinal symptoms can be controlled, the quality of live and longevity of cystic fibrosis patients is mainly determined by pulmonary inflammation and chronic pulmonary infections with Pseudomonas aeruginosa, Staphylococcus aureus, Burkholderia cepacia and Haemophilus influenzae. Recent studies developed novel and exciting concepts regarding the pathogenesis and treatment of cystic fibrosis. In particular, several studies indicated a critical role of death receptors, caveolae proteins, membrane rafts, alterations of the ceramide metabolism with an accumulation of ceramide and a reduction of 15-keto-prostaglandin 2 in cystic fibrosis lungs. These alterations have been found to be critically involved in the pulmonary inflammation and infection susceptibility of cystic fibrosis patients. However, albeit these studies provided novel insights into molecular mechanisms causing inflammation and vulnerability to infection, the details of these processes are still unknown.
囊性纤维化的特征是严重的肠道和肺部症状，但也会影响肝脏、胰腺和生殖道。由于胃肠道症状可以得到控制，囊性纤维化患者的生活质量和寿命主要取决于肺部炎症和慢性肺部感染，包括铜绿假单胞菌、金黄色葡萄球菌、洋葱伯克霍尔德菌和流感嗜血杆菌。最近的研究提出了囊性纤维化发病机制和治疗的新观点。特别是，几项研究表明，死亡受体、陷窝蛋白、膜筏、神经酰胺代谢的改变与神经酰胺的积累和 15-酮前列腺素 2 的减少在囊性纤维化肺中具有关键作用。这些改变被发现与囊性纤维化患者的肺部炎症和感染易感性密切相关。然而，尽管这些研究为导致炎症和易感染的分子机制提供了新的见解，但这些过程的细节仍不清楚。
