Orenstein D M, Bowen A
Department of Pediatrics, University of Pittsburgh School of Medicine, Pennsylvania.
Radiol Clin North Am. 1993 May;31(3):617-30.
The clinical features and current management of cystic fibrosis are reviewed, with emphasis on pulmonary manifestations. Pulmonary radiographic findings in cystic fibrosis are illustrated. The role of transplantation as a therapeutic avenue is discussed. Recent discoveries--the specific genetic mutation in cystic fibrosis, the elucidation of the cellular defect, and the development of gene-transfer techniques to correct the cellular defect in vitro--have generated fresh excitement in the search for an ultimate cure for cystic fibrosis.
本文综述了囊性纤维化的临床特征及当前治疗方法，重点关注肺部表现。文中展示了囊性纤维化的肺部影像学表现。讨论了移植作为一种治疗途径的作用。近期的发现——囊性纤维化的特定基因突变、细胞缺陷的阐明以及体外纠正细胞缺陷的基因转移技术的发展——在寻找囊性纤维化最终治愈方法的研究中引发了新的热潮。
