Winkler U, Wingender J, Jäger K E
Klin Wochenschr. 1985 Jun 3;63(11):490-8. doi: 10.1007/BF01747978.
The main cause of death in cystic fibrosis (CF) patients is progressive pulmonary insufficiency frequently associated with chronic infections of the respiratory tract by Pseudomonas aeruginosa. Bacteria of this species synthesize numerous extracellular products contributing to its pathogenicity. An alginate-like exopolysaccharide is characteristic for mucoid mutants predominating among P. aeruginosa isolates from CF patients. It interferes with immune defense mechanisms of the host and probably protects the bacteria against certain antibiotics. Furthermore, it is involved in the formation of bacterial microcolonies that resist mucociliary clearance, opsonisation, and phagocytosis. Exotoxin A and elastase are regarded as the most important among various extracellular enzymes involved in pulmonary injury in CF patients. Exotoxin A inhibits eukaryotic protein synthesis leading to necrosis; elastase, together with other Pseudomonas-proteases, induces hemorrhagic lesions and necrosis and seems to inactivate immunoglobulins and complement factors. Phospholipase C and glycolipid represent two hemolysins of P. aeruginosa that may contribute to cytopathogenic effects in infected lungs. No primary defect in the immunological defense mechanisms of CF patients has been described so far. Antibodies against various P. aeruginosa antigens including those mentioned above have been demonstrated, but a complete elimination of the bacteria from infected lungs has not been observed. Therapy of pulmonary P. aeruginosa infections in CF patients usually includes combinations of antibiotics of the beta-lactam and aminoglycoside type. Difficulties arise from an unusually high intrinsic resistance of P. aeruginosa as well as from poor penetration of many antibiotics into the sputum of CF patients. Therefore, future efforts to manage the Pseudomonas problem in CF will probably concentrate on prophylactic therapy, e.g. childhood vaccination of CF patients in order to prevent bacterial colonization of the respiratory tract.
囊性纤维化（CF）患者的主要死因是进行性肺功能不全，这常与铜绿假单胞菌引起的慢性呼吸道感染有关。该菌种的细菌能合成多种胞外产物，增强其致病性。一种类似藻酸盐的胞外多糖是黏液样突变体的特征，在从CF患者分离出的铜绿假单胞菌菌株中占主导地位。它干扰宿主的免疫防御机制，可能保护细菌免受某些抗生素的作用。此外，它还参与细菌微菌落的形成，这些微菌落可抵抗黏液纤毛清除、调理作用和吞噬作用。外毒素A和弹性蛋白酶被认为是CF患者肺部损伤相关的各种胞外酶中最重要的。外毒素A抑制真核生物蛋白质合成导致坏死；弹性蛋白酶与其他假单胞菌蛋白酶一起，可诱导出血性病变和坏死，似乎还能使免疫球蛋白和补体因子失活。磷脂酶C和糖脂是铜绿假单胞菌的两种溶血素，可能在受感染的肺部产生细胞致病作用。目前尚未发现CF患者免疫防御机制存在原发性缺陷。已证实存在针对包括上述各种铜绿假单胞菌抗原的抗体，但尚未观察到从感染肺部完全清除细菌的情况。CF患者肺部铜绿假单胞菌感染的治疗通常包括β-内酰胺类和氨基糖苷类抗生素联合使用。困难在于铜绿假单胞菌具有异常高的固有耐药性，以及许多抗生素难以渗透到CF患者的痰液中。因此，未来解决CF患者铜绿假单胞菌问题的努力可能会集中在预防性治疗上，例如对CF患儿进行疫苗接种，以防止呼吸道细菌定植。
