Long-Term Pulmonal Therapy of Cystic Fibrosis-Patients with Amitriptyline
Long-Term Pulmonal Therapy of Cystic Fibrosis-Patients with Amitriptyline
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DOI：
10.1159/000445648
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发表时间：
2016-01-01
影响因子：
--
通讯作者：
Riethmueller, Joachim
中科院分区：
文献类型：
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作者：
Adams, Constantin;Icheva, Vanya;Riethmueller, Joachim
背景/目标：最近的几项临床研究显示，神经酰胺在囊性纤维化（CF）患者的支气管上皮细胞中蓄积。使用功能性酸性鞘磷脂酶抑制剂阿米替林的CF患者肺中神经酰胺浓度的降解显示了肺功能、体重和加重率的益处。方法：为了测试阿米替林在体内的有益作用，我们进行了两项II期随机、双盲、安慰剂对照研究。CF患者用25 mg阿米替林每日两次治疗，即总剂量为50 mg/d。在这两项研究后，部分患者使用阿米替林作为常规治疗的非处方药。这些患者在连续使用阿米替林后1年、2年和3年进行观察，并与未接受治疗的患者进行匹配。这些患者被用作对照组。结果：治疗一年后，1秒用力呼气量预测值（FFV 1）显著增加7.6 ± 7.0%，p= 0.001。
Background/Aims: Several recent clinical studies revealed an accumulation of ceramide in bronchial epithelial cells of patients with cystic fibrosis (CF). Degradation of ceramide concentrations in lungs of CF patients employing the functional acid sphingomyelinase inhibitor amitriptyline revealed a benefit in lung function, weight and exacerbation rates. Methods: To test for a beneficial effect of amitriptyline in vivo, we performed two phase II randomised, double-blind, placebo-controlled studies. CF patients were treated with 25 mg amitriptyline twice daily, i.e. a total dose of 50 mg/d. After those two studies part of the patients used amitriptyline in an off-lable-use for routine treatment. These patients were observed after one, two and three years after continuous use of amitriptyline and were matched with those patients who were not treated. These patients were used as a control group. Results: After one year of treatment, forced expiratory volume in 1 sec predicted (FFV1) increased significantly by 7.6 +/- 7.0%, p=