文摘
Lymphangioleiomyomatosis (LAM)是一种罕见的低度恶性肿瘤,肺囊肿形成。一些进步的疾病患者需要肺移植(LT)。这里我们描述的临床特点和手术后结果LT LAM患者在巴西参考中心。我们回顾性研究包括11 LAM病人提交LT圣保罗大学从2003年到2016年双肺(91%)。意味着LT 44个月后随访。平均年龄在LT 43±7年。术前,患者出现呼吸困难(100%),补充氧气使用(91%),气胸(36%)和肺动脉高压(36%)。肋膜一个病人接受了,没有乳糜胸。西罗莫司之前使用的36%的患者FEV1 28±14%,中尉DLCO 30±18%的预测。没有术中并发症,而一个病人需要心肺旁路由于持续的血氧不足。 Post operatively, 18% of patients had chylothorax; 82% presented at least one episode of acute rejection, and chronic lung allograft syndrome occurred in 45%. At least one pulmonary infection occurred in 55% of patients, mainly due to Aspergillus sp. After LT, we found no LAM recurrence, episode of malignant neoplasm or retransplantation. There were 3 deaths (27%) and the survival rate at 5 years after LT was 77% (fig 1). Based on our data, LT is a feasible therapeutic option for patients with end-stage LAM, with favorable prognosis.
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