Unal-Maelger O H, Urbanek R
Universitäts-Kinderklinik Freiburg i. Br.
Monatsschr Kinderheilkd. 1988 Feb;136(2):76-80.
Patients with cystic fibrosis (CF) have an increased transepithelial electrical potential difference (PD) on the respiratory mucosa. In an attempt to investigate the diagnostic value of this measurement 12 patients with CF, 15 first degree relatives and 38 controls were studied. A nasal recording electrode and a reference electrode placed subcutaneously in the left upper arm were used. The recorded values of nasal PD were significantly (p less than 0.0001) higher (-61 mV +/- 7) than in subjects without cystic fibrosis (-21 mV +/- 6) and in CF relatives (-25 mV +/- 8). There was an overlap in values measured on the lip and on the tongue of subjects with and without cystic fibrosis. No differences in nasal PD were noted between male or female subjects. Transepithelial potential difference declined slightly with age. In addition, a superfusion with amiloride as inhibitor of sodium transport induced a 62% reduction in nasal PD in a cystic fibrosis patient as compared to a healthy subject (31%). These results as well as the good reproducibility (r = 0.99) demonstrate that the measurement of transepithelial potential difference on the nasal mucosa could be an additional diagnostic tool in cystic fibrosis.
囊性纤维化（CF）患者呼吸道黏膜的跨上皮电位差（PD）升高。为了研究该测量方法的诊断价值，对12例CF患者、15名一级亲属和38名对照者进行了研究。使用置于鼻腔的记录电极和皮下置于左上臂的参考电极。CF患者鼻腔PD的记录值（-61 mV±7）显著高于（p<0.0001）非CF患者（-21 mV±6）和CF亲属（-25 mV±8）。囊性纤维化患者与非囊性纤维化患者在唇部和舌部测量值存在重叠。男性和女性受试者鼻腔PD无差异。跨上皮电位差随年龄略有下降。此外，与健康受试者相比，用氨氯吡咪作为钠转运抑制剂进行灌流时，CF患者鼻腔PD降低62%，而健康受试者降低31%。这些结果以及良好的重复性（r = 0.99）表明，测量鼻腔黏膜跨上皮电位差可能是囊性纤维化的一种辅助诊断工具。
