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罕见病DIPNECH综合征的治疗：口服西罗莫司

2018-04-23 MedSci MedSci原创

DIPNECH综合征西罗莫司罕见病
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西罗莫司（中文别名：雷帕霉素）是一种新型大环内酯类免疫抑制剂，可用于预防肾移植后的排斥反应。但近日的研究表明，3例罕见弥漫性特发性肺神经内分泌细胞增生症（即DIPNECH综合征）经西罗莫司得到了成功治疗。

西罗莫司（中文别名：雷帕霉素）是一种新型大环内酯类免疫抑制剂，可用于预防肾移植后的排斥反应。但近日的研究表明，3例罕见弥漫性特发性肺神经内分泌细胞增生症（即DIPNECH综合征）经西罗莫司得到了成功治疗。

DIPNECH综合征是导致支气管壁神经内分泌细胞增殖和肿瘤形成的癌前病变，患者表现为隐匿性咳嗽或缓慢进展的呼吸困难，这些症状与细胞增殖和神经内分泌肽相关。近日，来自法国的研究小组发现三名患有DIPNECH综合征的女性（均出现呼吸困难），患者的症状用支气管扩张剂和吸入性皮质类固醇治疗后所有患者的肺功能均出现恶化，而类癌肿瘤的体积增加。该团队开始用口服型西罗莫司（2毫克/天）治疗患者，同时继续进行其他治疗。然而，由于患者不能忍受原始剂量的副作用，因此剂量降低至1至1.5毫克/天，在较低的剂量下，副作用问题得以解决。治疗1年后，3名患者的肺功能得到改善，CT扫描显示两名患者的结节体积和空气潴留均减少。


原始出处：

https://www.europeanpharmaceuticalreview.com/news/74369/oral-sirolimus-dipnech-syndrome/

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    2018-06-20 1249842em09(暂无昵称)

    #NEC#

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    2018-04-25 ms3415360956839622

    #西罗莫司#

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    2018-04-25 zhouqu_8

    #综合征#

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    2018-04-25 syscxl

    #罕见#

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