Bijman J
Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands.
Acta Paediatr Scand Suppl. 1989;363:10-3. doi: 10.1111/apa.1989.78.s363.10.
Studies of the disease CF suggest that the basic defect is related to impaired electrolyte movement in the epithelia of a variety of organs with exocrine function. The disturbances of electrolyte secretion in the organs classically involved in CF range from 1) a decrease in secretion- or uptake of chloride ions in all the organs studied; 2) an increase in sodium uptake in nasal airway epithelium and 3) a decrease in bicarbonate output of the pancreas. In this review an overview is presented of the expression of the CF defect, the abnormalities of fluid and electrolyte secretion in each CF affected organ are considered in more detail with particular emphasis on the hormonal and neuronal (dys)regulation of iron transport systems in epithelial cells.
对囊性纤维化（CF）疾病的研究表明，基本缺陷与具有外分泌功能的多种器官上皮中电解质转运受损有关。传统上认为与CF相关的器官中电解质分泌紊乱包括：1）在所研究的所有器官中氯离子分泌或摄取减少；2）鼻气道上皮中钠摄取增加；3）胰腺中碳酸氢盐分泌减少。在本综述中，概述了CF缺陷的表现，更详细地考虑了每个受CF影响器官中液体和电解质分泌的异常情况，特别强调了上皮细胞中铁转运系统的激素和神经元（失调）调节。
