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Sturge-Weber綜合征的視網膜血管異常

肺動脈肉瘤一例并文獻復習

目的 探討肺動脈肉瘤（pulmonary artery sarcoma，PAS）的臨床表現、影像學表現、病理學特點及鑒別診斷，提高臨床醫生對 PAS 的診治水平。方法 分析1 例經病理活檢確診的 PAS 患者的臨床資料并復習相關文獻。結果 患者男，72 歲，因“咳嗽、胸悶半年”于 2018 年 2 月 27 日首次入院。既往體健。查體無特殊。胸部 CT 示左肺下葉見多發結節狀高密度影，左肺動脈主干及分支內見斑塊狀低密度充盈影。診斷肺栓塞，予低分子肝素抗凝等治療后好轉出院。2018 年 4 月患者癥狀加重，復查 CT 肺動脈造影示左肺門增大，左肺動脈擴張，左肺動脈干管腔內巨大充盈缺損，肺動脈分叉處、左肺動脈多個分支及左心房亦可見多發充盈缺損，與首次入院時病灶相比明顯進展。正電子發射斷層顯像檢查示左肺動脈主干管腔擴張伴腔內低密度影，氟代脫氧葡萄糖代謝減低，左肺下葉結節樣放射性濃聚影，SUVmax 10.3。行經肺動脈介入抽吸取栓術，病理確診為 PAS。以“pulmonary artery sarcoma”“肺動脈肉瘤”“PAS”“pulmonary artery intimal sarcoma”“肺動脈內膜肉瘤”“PAIS”“pulmonary thromboembolism”“肺栓塞”“PTE”為關鍵詞對國內外文獻進行檢索，共檢索到相關文獻 10 篇，涉及 506 例患者，其中男 265 例，女 241 例。PAS 患者的主要癥狀為咳嗽和胸悶。CT 肺動脈造影見肺動脈主干充盈缺損，可累及左右肺動脈及其分支。PAS 大都經手術確診。病理見黏液背景中大量類似成纖維細胞或肌成纖維細胞的梭形細胞肉瘤，核異型性及有絲分裂像明顯。免疫組織化學檢測無特異性標志物，波形蛋白強陽性，CD31、CD34、Fli-1 不同程度陽性。手術切除是其主要的治療手段，預后差。結論 PAS 是罕見的肺血管系統的惡性腫瘤。患者的臨床表現、影像學檢查和病理免疫組織化學檢測無明顯特異性，常被誤診為 PTE。及時診斷和早期治療是延長患者生存時間的關鍵。

Research on the protective effect and mechanisms of 4-phenylbutyric acid on bleomycin-induced pulmonary fibrosis in mice

Objective To explore the effects of 4-phenylbutyric acid (4-PBA) on idiopathic pulmonary fibrosis (IPF) using a murine model of bleomycin (BLM)-induced pulmonary fibrosis. Methods Pulmonary fibrosis was induced in C57BL/6 mice by intratracheal injection of BLM. A total of 120 mice were randomly allocated into three groups: BLM group, BLM+4-PBA group, and control group. Pathology of lung tissue was analyzed to evaluate the degree of pulmonary fibrosis, and the survival of the mice was noted. The expression levels of the endoplasmic reticulum stress markers, activating transcription factor 6 (ATF6) and C/EBP homologous protein (CHOP), were analyzed in lung tissues from mice. Results BLM induced significant collagen deposition in the lungs of the mice, which was alleviated by 4-PBA. 4-PBA also dramatically improved the pulmonary function and increased the survival rate in the BLM+4-PBA group compared with that in the BLM group. Both the mRNA and protein expression levels of ATF6 and CHOP were significantly reduced in mouse lung tissue after 2 weeks of 4-PBA treatment. Conclusions 4-PBA treatment could alleviate BLM-induced pulmonary fibrosis in mice via the attenuation of endoplasmic reticulum stress.