College of Pharmacy, Department of Pharmacy Practice, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA.
Ann Pharmacother. 2010 Mar;44(3):577-81. doi: 10.1345/aph.1M642. Epub 2010 Feb 23.
To describe the use of lubiprostone for constipation in 3 adults with cystic fibrosis (CF).
This case series describes the use of lubiprostone for the treatment of constipation in 3 adults with CF (mean +/- SD length of therapy 17.3 +/- 1.5 mo). All 3 patients were prescribed lubiprostone 24 microg twice daily after hospitalization for treatment of intestinal obstruction. Patient 1 continues on chronic polyethylene glycol (PEG) 3350 and lubiprostone and has not had a recurrence of obstruction. Patient 2 requires aggressive chronic therapy with PEG 3350, lubiprostone, and methylnaltrexone. She has had 1 recurrence of obstruction. Patient 3 continues with lubiprostone taken several times per week with good control of constipation and no recurrence of obstruction to date. The adverse effect profile has been tolerable in all 3 patients.
CF is caused by a genetic mutation resulting in a dysfunctional or absent CF transmembrane conductance regulator that normally functions as a chloride channel. This results in viscous secretions in multiple organ systems including the lungs and intestinal tract. Accumulation of viscous intestinal contents contributes to constipation, which is common among adults with CF and can sometimes lead to intestinal obstruction. Lubiprostone is indicated for chronic constipation and works by activating type 2 chloride channels (ClC-2) in the intestinal tract. Because it utilizes an alternate chloride channel, lubiprostone may be especially effective for constipation in patients with CF.
Lubiprostone provides an additional option for the treatment of constipation in adults with CF. Its use in the CF population deserves further study.
描述在 3 例囊性纤维化（CF）成人中使用鲁比前列酮治疗便秘的情况。
本病例系列描述了 3 例 CF 成人使用鲁比前列酮治疗便秘的情况（平均治疗时长为 17.3 +/- 1.5 个月）。所有 3 例患者在因肠梗阻住院后均被开处鲁比前列酮 24 微克，每日两次。患者 1 继续接受慢性聚乙二醇（PEG）3350 和鲁比前列酮治疗，未再发生肠梗阻。患者 2 需要积极接受慢性 PEG 3350、鲁比前列酮和甲基纳曲酮治疗。她曾发生 1 次肠梗阻复发。患者 3 继续每周服用数次鲁比前列酮，便秘得到很好的控制，且迄今未再发生肠梗阻。所有 3 例患者的不良反应谱均可耐受。
CF 由基因突变引起，导致 CF 跨膜电导调节蛋白功能异常或缺失，而该蛋白通常作为氯离子通道发挥作用。这会导致包括肺部和肠道在内的多个器官系统产生粘性分泌物。粘性肠道内容物的积累导致便秘，这在 CF 成人中很常见，有时可导致肠梗阻。鲁比前列酮用于慢性便秘，通过激活肠道中的 2 型氯离子通道（ClC-2）发挥作用。由于它利用了替代氯离子通道，鲁比前列酮可能对 CF 患者的便秘特别有效。
鲁比前列酮为 CF 成人的便秘治疗提供了另一种选择。它在 CF 人群中的应用值得进一步研究。
