Windstetter D, Schaefer F, Schärer K, Reiter K, Eife R, Harms H K, Bertele-Harms R, Fiedler F, Tsui L C, Reitmeir P, Horster M, Hadorn H B
Department of Neonatology, Universitätsklinikum Charité der Humboldt Universität Schumannstr. 20/21 Berlin D-10098 Germany +49 30/2802 5650 +49 30/2802 5824.
Eur J Med Res. 1997 Oct 30;2(10):431-6.
In ten cystic fibrosis patients and nine age-matched controls, renal function was determined before and after infusion of secretin. Under baseline conditions creatinine excretion and clearance were significantly elevated, exclusively due to those patients who were homozygous for the DF508 mutation (153 vs 132 ml/min1.73m2), whereas the glomerular filtration rate, measured by inulin clearance showed no difference. Renal plasma flow and the fractional reabsorption rates of electrolytes were similar in patients and controls. During secretin infusion renal plasma flow increased and the fractional reabsorption rates of electrolytes decreased in both groups. The patients had a increased metabolic clearance (2900 vs 1660 ml/minm2) and endogenous production rate (9,9 vs 2,5 pmol/min*m2) of of secretin. In conclusion global renal function and electrolyte handling, in particular chloride permeability, are unchanged in cystic fibrosis. Individuals expressing the DF508 genotype showed a selective elevation of creatinine excretion and clearance. The secretion and metabolic clearance of secretin are increased in cystic fibrosis.
在10名囊性纤维化患者和9名年龄匹配的对照者中，在注射促胰液素前后测定了肾功能。在基线条件下，肌酐排泄和清除率显著升高，这完全是由于那些DF508突变纯合子患者（153对132 ml/min1.73m2），而通过菊粉清除率测量的肾小球滤过率没有差异。患者和对照者的肾血浆流量以及电解质的分数重吸收率相似。在注射促胰液素期间，两组的肾血浆流量均增加，电解质的分数重吸收率均降低。患者的促胰液素代谢清除率（2900对1660 ml/minm2）和内源性产生率（9.9对2.5 pmol/min*m2）增加。总之，囊性纤维化患者的整体肾功能和电解质处理，特别是氯离子通透性没有改变。表达DF508基因型的个体肌酐排泄和清除率选择性升高。囊性纤维化患者促胰液素的分泌和代谢清除率增加。
