Michalsen H, Monn E, Bergan T, Churg J, Johannessen J V
Pathol Res Pract. 1984 Jan;178(3):261-7. doi: 10.1016/S0344-0338(84)80108-9.
Pharmacokinetic studies on antibiotics as well as other studies on renal function indicate renal malfunction in cystic fibrosis. Renal biopsies from two children, and post mortem examination from one child, with this disorder were normal by light microscopy and close to normal by immunofluorescence examination and transmission and scanning electron microscopy, even in cases with advanced disease and marked renal malfunction. Renal biopsy interpretation thus seems to be of little value in the evaluation of renal malfunction in this disorder, and this suggests that the functional abnormalities are caused by metabolic/biochemical defects.
抗生素的药代动力学研究以及其他关于肾功能的研究表明，囊性纤维化患者存在肾功能障碍。对两名患有这种疾病的儿童进行肾活检，以及对一名患有这种疾病的儿童进行尸检，结果显示，通过光学显微镜检查，肾脏正常；通过免疫荧光检查、透射电子显微镜检查和扫描电子显微镜检查，肾脏接近正常，即使在疾病晚期且存在明显肾功能障碍的情况下也是如此。因此，肾活检结果在评估这种疾病的肾功能障碍方面似乎价值不大，这表明功能异常是由代谢/生化缺陷引起的。
