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[氨氯吡咪吸入疗法治疗黏多糖病]

[Amiloride inhalation therapy in mucoviscidosis].

作者信息

Lindemann H

机构信息

Universitäts-Kinderklinik Giessen.

出版信息

Monatsschr Kinderheilkd. 1993 May;141(5):392-4.

PMID:8326957
Abstract

The defective regulation of the secretory chloride channel and the accelerated rate of sodium absorption in the airway epithelia of patients with cystic fibrosis (CF) contribute to the dehydration of airway secretions which is responsible for secondary damage in the respiratory tract. The sodium-blocker amiloride inhibits sodium absorption depending on the dosage of the drug. This effect is documented by means of measuring transepithelial potential difference which is decreased following to inhalative amiloride administration in CF patients. Clinical long term efficiency has been shown to be probable, but has to be confirmed. Investigation in this field is difficult, because there are some other important factors which influence viscosity of mucus. The use of amiloride can best be evaluated, if it is given immediately after birth. However, the answer to some important questions has to be found before.

摘要

囊性纤维化(CF)患者气道上皮中分泌性氯离子通道的调节缺陷以及钠吸收速率加快,导致气道分泌物脱水,这是呼吸道继发性损伤的原因。钠阻滞剂氨氯地平根据药物剂量抑制钠吸收。通过测量跨上皮电位差来记录这种作用,CF患者吸入氨氯地平后跨上皮电位差会降低。临床长期疗效已显示有可能,但有待证实。该领域的研究很困难,因为还有其他一些重要因素会影响黏液的黏度。如果在出生后立即给予氨氯地平,其使用效果最佳。然而,在此之前必须找到一些重要问题的答案。

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