Vawter G F, Shwachman H
Pathol Annu. 1979;14 Pt 2:357-82.
An annotated catalog of clinical and pathologic observations in an adult population with CF has been presented. The spectrum of disease is broad, and the diagnosis usually requires the demonstration of abnormalities in multiple systems. Tissues of each germ layer have been found involved in CF. Correlation of clinical and pathologic observations suggests that manifestations are essentially episodic and focal. In this population, the most characteristic abnormalities recognized by the pathologist are focal biliary cirrhosis, distinctive obstructive lesions of the male genital tract, prolonged staphylococcal or pseudomonas colonization of respiratory secretions, and obstructive bronchopulmonary disease. Some form of pancreatic atrophy is usually present.
一份关于成年囊性纤维化（CF）患者临床和病理观察结果的注释目录已经公布。疾病谱广泛，诊断通常需要证明多个系统存在异常。已发现每个胚层的组织都参与了CF。临床和病理观察结果的相关性表明，表现基本上是发作性和局灶性的。在这一人群中，病理学家识别出的最具特征性的异常是局灶性胆汁性肝硬化、男性生殖道独特的阻塞性病变、呼吸道分泌物长期被葡萄球菌或假单胞菌定植以及阻塞性支气管肺疾病。通常存在某种形式的胰腺萎缩。
