Use of the Chloride Channel Activator Lubiprostone for Constipation in Adults with Cystic Fibrosis: A Case Series
Use of the Chloride Channel Activator Lubiprostone for Constipation in Adults with Cystic Fibrosis: A Case Series
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DOI：
10.1345/aph.1m642
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发表时间：
2010-03-01
影响因子：
2.9
通讯作者：
Stowe, Cindy D.
中科院分区：
文献类型：
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作者：
O'Brien, Catherine E.;Anderson, Paula J.;Stowe, Cindy D.
目的：描述鲁比前列酮在3名患有囊性纤维化（CF）的成人便秘患者中的应用。
病例总结：本病例系列描述了鲁比前列酮在3名CF成人患者便秘治疗中的应用（平均±标准差治疗时长为17.3±1.5个月）。这3名患者均在因肠梗阻住院治疗后被处方鲁比前列酮，剂量为每日两次，每次24微克。患者1继续使用慢性聚乙二醇（PEG）3350和鲁比前列酮，未再出现肠梗阻复发。患者2需要积极的慢性治疗，使用PEG 3350、鲁比前列酮和甲基纳曲酮，她出现过1次肠梗阻复发。患者3继续每周使用数次鲁比前列酮，便秘得到良好控制，截至目前未出现肠梗阻复发。这3名患者对不良反应均耐受。
讨论：CF是由基因突变导致囊性纤维化跨膜传导调节因子功能失调或缺失引起的，该调节因子通常作为氯离子通道发挥作用。这导致包括肺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聚导致便秘，便秘在CF成人患者中很常见，有时可导致肠梗阻。鲁比前列酮用于治疗慢性便秘，通过激活肠道中的2型氯离子通道（CIC - 2）起作用。由于它利用了一种替代的氯离子通道，鲁比前列酮可能对CF患者的便秘特别有效。
结论：鲁比前列酮为CF成人患者的便秘治疗提供了一种额外的选择。它在CF人群中的应用值得进一步研究。
OBJECTIVE: To describe the use of lubiprostone for constipation in 3 adults with cystic fibrosis (CF).CASE SUMMARY: 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 mu g 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.DISCUSSION: 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 (CIC-2) in the intestinal tract. Because it utilizes an alternate chloride channel, lubiprostone may be especially effective for constipation in patients with CF.CONCLUSIONS: Lubiprostone provides an additional option for the treatment of constipation in adults with CF. Its use in the CF population deserves further study.