Namba Masashi, Masuda Takeshi, Nakamura Takashi, Horimasu Yasushi, Miyamoto Shintaro, Nakashima Taku, Iwamoto Hiroshi, Fujitaka Kazunori, Hamada Hironobu, Hattori Noboru
Department of Respiratory Internal Medicine, Hiroshima University Hospital, Japan.
Postgraduate Medical and Dental Training Center, Hiroshima University Hospital, Japan.
Intern Med. 2017 Dec 15;56(24):3327-3331. doi: 10.2169/internalmedicine.8645-16. Epub 2017 Oct 11.
Recently, sirolimus, an inhibitor of mammalian target of rapamycin, was reported to decrease chylous effusion in patients with lymphangioleimyomatosis (LAM). We herein report a case of a 34-year-old woman with LAM who developed refractory chylothorax and chylous ascites during sirolimus therapy. In this case, to reduce chylous effusion, we administered octreotide, which is often used to control postoperative chylous effusion, in addition to the sirolimus therapy. This combination therapy reduced the chylothorax and chylous ascites. For patients with LAM, octreotide therapy in addition to sirolimus may be effective for treating sirolimus-refractory chylous effusion.
最近,有报道称雷帕霉素(一种哺乳动物雷帕霉素靶蛋白抑制剂)可减少淋巴管平滑肌瘤病(LAM)患者的乳糜性积液。我们在此报告一例34岁的LAM女性患者,其在接受雷帕霉素治疗期间出现了难治性乳糜胸和乳糜性腹水。在该病例中,为减少乳糜性积液,除雷帕霉素治疗外,我们还给予了常用于控制术后乳糜性积液的奥曲肽。这种联合治疗减少了乳糜胸和乳糜性腹水。对于LAM患者,除雷帕霉素外,奥曲肽治疗可能对治疗雷帕霉素难治性乳糜性积液有效。