联合辅酶 Q10、左旋肉碱和阿米替林治疗周期性呕吐综合征的高度疗效：病例系列研究。
High degree of efficacy in the treatment of cyclic vomiting syndrome with combined co-enzyme Q10, L-carnitine and amitriptyline, a case series.
机构信息
Division of Medical Genetics and the Saban Research Institute, Children's Hospital Los Angeles, California, USA.
出版信息
BMC Neurol. 2011 Aug 16;11:102. doi: 10.1186/1471-2377-11-102.
BACKGROUND
Cyclic vomiting syndrome (CVS), defined by recurrent stereotypical episodes of nausea and vomiting, is a relatively-common disabling and historically difficult-to-treat condition associated with migraine headache and mitochondrial dysfunction. Limited data suggests that the anti-migraine therapies amitriptyline and cyproheptadine, and the mitochondrial-targeted cofactors co-enzyme Q10 and L-carnitine, have efficacy in episode prophylaxis.
METHODS
A retrospective chart review of 42 patients seen by one clinician that met established CVS diagnostic criteria revealed 30 cases with available outcome data. Participants were treated on a loose protocol consisting of fasting avoidance, co-enzyme Q10 and L-carnitine, with the addition of amitriptyline (or cyproheptadine in those < 5 years) in refractory cases. Blood level monitoring of the therapeutic agents featured prominently in management.
RESULTS
Vomiting episodes resolved in 23 cases, and improved by > 75% and > 50% in three and one additional case respectively. Among the three treatment failures, two could not tolerate amitriptyline (as was also the case in the child with only > 50% efficacy) and one had multiple congenital gastrointestinal anomalies. Excluding the latter case, substantial efficacy (> 75% response) was 26/29 at the start of treatment, and 26/26 in those able to tolerate the regiment, including high dosages of amitriptyline.
CONCLUSION
Our data suggest that a protocol consisting of mitochondrial-targeted cofactors (co-enzyme Q10 and L-carnitine) plus amitriptyline (or possibly cyproheptadine in preschoolers) coupled with blood level monitoring is highly effective in the prevention of vomiting episodes.
背景
周期性呕吐综合征（CVS）定义为反复出现刻板的恶心和呕吐发作，是一种相对常见的致残性疾病，既往治疗困难，与偏头痛和线粒体功能障碍有关。有限的数据表明，抗偏头痛治疗药物阿米替林和赛庚啶，以及线粒体靶向辅因子辅酶 Q10 和左旋肉碱，在发作预防方面具有疗效。
方法
对一位临床医生诊治的符合既定 CVS 诊断标准的 42 例患者进行回顾性图表审查，其中 30 例有可用的结局数据。根据一项宽松的方案对参与者进行治疗，方案包括禁食避免、辅酶 Q10 和左旋肉碱，对于难治性病例，在添加阿米替林（或对于< 5 岁的患者添加赛庚啶）。治疗药物的血药浓度监测在管理中占有重要地位。
结果
23 例患者呕吐发作得到缓解，3 例患者改善程度> 75%，1 例患者改善程度> 50%。在 3 例治疗失败的患者中，2 例不能耐受阿米替林（与仅> 50%疗效的儿童患者情况相同），1 例患者存在多种先天性胃肠道异常。排除后者，在开始治疗时，有 26/29 例患者的疗效显著（> 75%的反应），而在能够耐受方案的 26/26 例患者中，包括阿米替林的高剂量治疗，也有显著疗效。
结论
我们的数据表明，由线粒体靶向辅因子（辅酶 Q10 和左旋肉碱）加用阿米替林（或可能在学龄前儿童中加用赛庚啶）组成的方案，并结合血药浓度监测，在预防呕吐发作方面非常有效。
Zotero 插件