Winklhofer-Roob B M
Department of Pediatrics, University of Zurich, Switzerland.
Acta Paediatr Suppl. 1994 Apr;83(395):49-57. doi: 10.1111/j.1651-2227.1994.tb13229.x.
Patients with cystic fibrosis frequently exhibit increased oxygen free radical generation from activated neutrophils due to chronic lung inflammation on the one hand and antioxidant deficiencies due to exocrine pancreatic insufficiency on the other, resulting in an oxidant-antioxidant imbalance in favor of the former. As a consequence, free radical attack on unsaturated fatty acids of lipid structures leading to lipid peroxidation and damaging effects on proteins may occur. In the lung, antiproteases are thought to be inactivated by oxygen free radicals released from inflammatory cells. In the cholestatic liver, bile acids may propagate lipid peroxidation. An efficient antioxidant supply is suggested to control tissue injury by restoring the oxidant-antioxidant balance. Mechanisms involved in the generation of oxygen free radicals are described and data on the antioxidant defense system in cystic fibrosis patients are presented, together with evidence of increased lipid peroxidation. Possible implications for disease processes are discussed as well as therapeutic concepts to reconstitute the oxidant-antioxidant balance.
一方面，由于慢性肺部炎症，囊性纤维化患者的活化中性粒细胞常常会产生更多的氧自由基；另一方面，由于外分泌性胰腺功能不全，患者存在抗氧化剂缺乏的情况，这导致了有利于前者的氧化还原失衡。结果，自由基会攻击脂质结构中的不饱和脂肪酸，导致脂质过氧化，并对蛋白质产生破坏作用。在肺部，抗蛋白酶被认为会被炎症细胞释放的氧自由基灭活。在胆汁淤积性肝脏中，胆汁酸可能会促进脂质过氧化。建议通过恢复氧化还原平衡来有效供应抗氧化剂，以控制组织损伤。本文描述了氧自由基生成所涉及的机制，介绍了囊性纤维化患者抗氧化防御系统的数据，以及脂质过氧化增加的证据。还讨论了对疾病进程的可能影响以及重建氧化还原平衡的治疗理念。
