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2em;">2.有壁，壁厚不一，通常均勻且＜2mm</p><p style="text-align: justify; text-indent: 2em;">3.通常含氣，偶爾含液或?qū)嵭猿煞?/p><p style="text-align: justify; text-indent: 2em;">4.需要鑒別疾病：肺大泡、肺氣腫、支氣管擴(kuò)張、空洞和肺氣囊、蜂窩樣改變</p><p style="text-align: justify; text-indent: 2em;"><strong>彌漫性肺囊性病變可分為：</strong></p><table class="a4" width="820"><tbody><tr class="firstRow"><td bgcolor="#FFFFFF" width="86"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #000000;">分類</span></p></td><td bgcolor="#FFFFFF" width="368"><p> </p></td></tr><tr><td bgcolor="#FFFFFF" width="86"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #000000;">腫瘤性</span></p></td><td bgcolor="#FFFFFF" width="368"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #FF0000;">肺淋巴管平滑肌瘤?。?/span><span style="color: #FF0000;">LAM</span><span style="color: #FF0000;">）、朗格漢斯組織細(xì)胞增生癥（</span><span style="color: #FF0000;">LCH</span><span style="color: #FF0000;">）</span><span style="color: #000000;">、肉瘤、腺癌、胸膜肺母細(xì)胞瘤</span></p></td></tr><tr><td bgcolor="#FFFFFF" width="86"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #000000;">先天</span><span style="color: #000000;">/</span><span style="color: #000000;">遺傳</span><span style="color: #000000;">/</span><span style="color: #000000;">發(fā)育性</span></p></td><td bgcolor="#FFFFFF" width="368"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #FF0000;">BHD</span><span style="color: #FF0000;">綜合癥</span><span style="color: #000000;">、</span><span style="color: #000000;">P</span><span style="color: #000000;">roteus</span><span style="color: #000000;">綜合征、神經(jīng)纖維瘤、支氣管發(fā)育不全、先天性肺氣道畸形</span></p></td></tr><tr><td bgcolor="#FFFFFF" width="86"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #000000;">淋巴組織增生性</span></p></td><td bgcolor="#FFFFFF" width="368"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #FF0000;">淋巴細(xì)胞性間質(zhì)性肺炎（</span><span style="color: #FF0000;">LIP</span><span style="color: #FF0000;">）</span><span style="color: #000000;">、濾泡性細(xì)支氣管炎、干燥綜合征、淀粉樣變性、輕鏈沉積病</span></p></td></tr><tr><td bgcolor="#FFFFFF" width="86"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #000000;">感染性</span></p></td><td bgcolor="#FFFFFF" width="368"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #FF0000;">耶氏肺孢子菌?。?/span><span style="color: #FF0000;">PJP</span><span style="color: #FF0000;">）</span><span style="color: #000000;">、葡萄球菌肺炎、地方性真菌病、肺吸蟲病</span></p></td></tr><tr><td bgcolor="#FFFFFF" width="86"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #000000;">間質(zhì)性肺疾病相關(guān)性</span></p></td><td bgcolor="#FFFFFF" width="368"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #000000;">過敏性肺炎、脫屑性間質(zhì)性肺?。?/span><span style="color: #000000;">DIP</span><span style="color: #000000;">）</span></p></td></tr><tr><td bgcolor="#FFFFFF" width="86"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #000000;">其他</span></p></td><td bgcolor="#FFFFFF" width="368"><p style="margin-top: 0; margin-bottom: 0;"><span style="color: #000000;">創(chuàng)傷后假性囊狀、</span><span style="color: #000000;">Fire-eater's</span><span style="color: #000000;"> </span><span style="color: #000000;">lung</span><span style="color: #000000;">、</span><span style="color: #000000;">Hyper-IgE</span><span style="color: #000000;"> </span><span style="color: #000000;">syndrome</span></p></td></tr></tbody></table><p style="margin-top: 0px; margin-bottom: 0px; text-align: justify; text-indent: 2em;"><span style="font-family: '仿宋_GB2312'; font-size: 16pt;">看到這，是不是完全暈了，別急！肺科間質(zhì)肺團(tuán)隊(duì)用真實(shí)的病例手把手來教你！</span></p><p style="margin-top: 0px; margin-bottom: 0px; text-align: justify; text-indent: 2em;"><span style="font-family: '黑體'; font-size: 16pt;">病例1</span><span style="font-family: '黑體'; font-size: 16pt;">：</span><span style="font-family: '仿宋_GB2312'; font-size: 16pt;">患者陳某，女，44歲，因“反復(fù)多次氣胸”就診我院，既往左腎錯構(gòu)瘤術(shù)后病史。</span></p><p style="text-align:center"><img src="/ueditor/php/upload/image/20240207/1707293126146562.png" width="757" height="223" title="1707293126146562.png" alt="微信圖片_20240207120513.png"/></p><p style="text-indent: 0em; text-align: center;"><strong>氣管鏡病理結(jié)果符合淋巴管平滑肌瘤病</strong></p><p style="text-indent: 2em;"><strong>肺淋巴管平滑肌瘤?。↙AM）</strong></p><p style="text-indent: 2em;">●LAM是一種罕見疾病，病因不明的平滑肌彌漫增生性全身性疾病，導(dǎo)致進(jìn)行性肺囊性變</p><p style="text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>多見于育齡女性，平均發(fā)病年齡為35歲</p><p style="text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>自發(fā)性氣胸的患病率最高，大約10%的<span style="text-indent: 2em;">LAM</span><span style="text-indent: 2em;">患者會出現(xiàn)乳糜</span><span style="text-indent: 2em;">胸</span></p><p style="text-indent: 2em;"><span style="text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>典型</span><span style="text-indent: 2em;">的影像表現(xiàn)為</span><span style="text-indent: 2em;">雙肺彌漫、薄壁、圓形或橢圓形、</span><span style="text-indent: 2em;">2-10mm</span><span style="text-indent: 2em;">的含氣囊腔，大者也可達(dá)</span><span style="text-indent: 2em;">30mm</span><span style="text-indent: 2em;">，壁薄，界限清楚，鄰近肺組織正常。</span></p><p style="text-indent: 2em;"><span style="font-family: '黑體'; font-size: 16pt;"><br/></span></p><p style="text-align: justify; text-indent: 2em;">病例2：羅某，男性，34歲，咳嗽4個月。2023年2月8日入院。個人史：吸煙17年，1包/日，未戒煙。</p><p style="text-align:center"><img src="/ueditor/php/upload/image/20240207/1707293192961241.png" width="800" height="188" title="1707293192961241.png" alt="微信圖片_20240207121529.png"/></p><p style="text-align: center;">氣管鏡肺活檢及肝穿刺活檢術(shù)，病理均提示朗格漢斯組織細(xì)胞增生癥</p><p style="text-align: justify; text-indent: 2em;">朗格漢斯組織細(xì)胞增生癥（LCH）</p><p style="text-align: justify; text-indent: 2em;">●LCH是一組罕見的單核-巨噬細(xì)胞異常增生性疾病，好發(fā)部位依次為骨骼、肺、垂體、皮膚、眼、肝、淋巴結(jié)、心臟和骨髓等</p><p style="text-align: justify; text-indent: 2em;">●發(fā)生機(jī)制與吸煙和BRAFV600E突變導(dǎo)致MAPK通路激活有關(guān)</p><p style="text-align: justify; text-indent: 2em;">●好發(fā)于年輕人（20-40歲）、與吸煙相關(guān)</p><p style="text-align: justify; text-indent: 2em;">●臨床表現(xiàn)常常局限于肺，主要表現(xiàn)為咳嗽、活動后氣短、氣胸</p><p style="text-align: justify; text-indent: 2em;"><span style="text-indent: 2em;">●</span><span style="text-indent: 2em;">典型的影像特征為囊腔形態(tài)奇異、不規(guī)則，呈分葉狀（三葉草）、壁厚或薄，合并結(jié)節(jié)及空洞，好發(fā)于上、中肺，很少累及肋膈角。</span></p><p style="margin-top: 0; margin-bottom: 0;"><span style="font-family: '黑體'; font-size: 16pt;"><br/></span></p><p style="text-align: justify; text-indent: 2em;">病例3：患者男，40歲，農(nóng)民，因“胸悶10余天”入住我院?；颊哂诎朐虑俺霈F(xiàn)左側(cè)氣胸，于當(dāng)?shù)蒯t(yī)院行左側(cè)胸腔閉式引流術(shù)，氣胸好轉(zhuǎn)后拔管出院。母親、舅舅有“肺大泡”、氣胸病史。</p><p></p><p style="text-align:center"><img src="/ueditor/php/upload/image/20240207/1707293800868201.png" width="760" height="299" title="1707293800868201.png" alt="微信圖片_20240207121606.png"/></p><p style="text-align: center; text-indent: 2em;">全基因組測序提示FLCN基因陽性，突變位點(diǎn)為c1538+3A>C。其母親存在相同的突變位點(diǎn)，診斷“BHD綜合征”</p><p style="text-align: justify; text-indent: 2em;"><strong>BHD綜合征</strong></p><p style="text-align: justify; text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>罕見、常染色體顯性遺傳、FLCN基因突變，多器官系統(tǒng)疾病</p><p style="text-align: justify; text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>臨床表現(xiàn):以皮膚纖維毛囊瘤，肺囊腔和腎腫瘤（嫌色細(xì)胞腺瘤和嗜酸細(xì)胞腺瘤最常見，也可是腎癌;雙側(cè)、多灶)為特征，75%患者中出現(xiàn)氣胸</p><p style="text-align: justify; text-indent: 2em;"><span style="text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>多見于30-50歲</span></p><p style="text-align: justify; text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>影像特點(diǎn)：囊圈少，呈圓/橢圓形，薄壁、大小不一，分布于肺底部、胸膜下區(qū)和縱隔旁</p><p style="text-align: justify; text-indent: 2em;"><br/></p><p style="text-align: justify; text-indent: 2em;">病例4：患者洪某某，男，63歲，以“咳嗽、咳痰、氣促9年余，加劇2周”為主訴入院。</p><p style="text-align:center"></p><p style="text-align: center; margin-top: 0; margin-bottom: 0;"><img src="/ueditor/php/upload/image/20240207/1707293878884070.png" width="897" height="273" title="1707293878884070.png" alt="微信圖片_20240207121812.png"/></p><p style="text-align:center"><img src="/ueditor/php/upload/image/20240207/1707293887831598.png" width="862" height="390" title="1707293887831598.png" alt="微信圖片_20240207121755.png"/></p><p style="margin-top: 0px; margin-bottom: 0px; text-align: center;"><span style="font-family: '黑體'; font-size: 16pt;">診斷：干燥綜合征、淋巴細(xì)胞間質(zhì)性肺炎（LIP）</span></p><p style="text-align: justify; text-indent: 2em;">淋巴細(xì)胞間質(zhì)性肺炎（LIP）</p><p style="text-align: justify; text-indent: 2em;">●特發(fā)性或繼發(fā)于自身免疫性疾病，多數(shù)與結(jié)締組織疾病有關(guān)如干燥綜合征SS (最常見)、RA、SLE、AIDS等。</p><p style="text-align: justify; text-indent: 2em;">●影像特征：囊腔少，較大，可達(dá)3cm，呈薄壁、隨機(jī)分布 (下葉多見) ，內(nèi)部可有分隔，近血管旁，伴支氣管血管束周圍間質(zhì)及小葉間隔增厚</p><p style="text-align: justify; text-indent: 2em;">看完以上4個病例，我們簡單總結(jié)下彌漫性肺囊性病變的診斷思路：是<span style="text-indent: 2em;">否是真的囊腔？</span><span style="text-indent: 2em;">是否是胸膜下囊腔？</span><span style="text-indent: 2em;">是否是肺實(shí)質(zhì)內(nèi)單發(fā)還是多發(fā)或彌漫囊腔？</span><span style="text-indent: 2em;">囊腔是否伴發(fā)結(jié)節(jié)？</span><span style="text-indent: 2em;">囊腔是否伴發(fā)</span><span style="text-indent: 2em;">膜</span><span style="text-indent: 2em;">玻璃影？</span></p><p style="text-align:center"><img border="0" style="width: 1061px; height: 602px;" src="/ueditor/php/upload/image/20240207/160401429png"/></p><p style="text-align: justify; text-indent: 2em;">我們也可以根據(jù)患者的性別、年齡、吸煙史、家族史等進(jìn)行大概的推斷：</p><p style="text-align:center"><img border="0" style="width: 680px; height: 620px;" src="/ueditor/php/upload/image/20240207/160401971png"/></p><p style="text-align: justify; text-indent: 2em;">看完小編的介紹，對于肺囊性病變?nèi)绻€覺得迷茫，那再看看最后的干貨總結(jié)：</p><p style="text-align: justify; text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>囊性肺疾病代表了一組不同的疾病，其共同的影像學(xué)特征是有壁（厚度≤2毫米）包圍的多個充滿空氣的透明層。彌漫性囊性肺疾病必須通過放射學(xué)檢查與肺氣腫、蜂窩狀、囊性支氣管擴(kuò)張、肺空洞和氣囊進(jìn)行鑒別。</p><p style="text-align: justify; text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>四種最常見的囊性肺病是淋巴管平滑肌瘤?。↙AM）、肺朗格漢斯細(xì)胞組織細(xì)胞增多癥（PLCH）、Birt-Hogg-Dubé綜合征（BHD）和淋巴樣間質(zhì)性肺炎（LIP）。</p><p style="text-align: justify; text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>在初步評估中，詳細(xì)的病史以及人口統(tǒng)計(jì)學(xué)（年齡、性別和種族）、吸煙史和家族史（例如結(jié)節(jié)性硬化癥 [TSC] 或 BHD 的特征）非常重要。仔細(xì)尋找肺外特征，包括皮膚、胸膜和腹腔內(nèi)表現(xiàn)，可以深入了解診斷。</p><p style="text-align: justify; text-indent: 2em;"><span style="text-indent: 32px; text-wrap: wrap;">●</span>高分辨率 CT 是評估過程的重要組成部分，通?？梢詤^(qū)分各種囊性肺疾病，特別是由影像科專家<span style="text-indent: 2em;">參與</span><span style="text-indent: 2em;">時。</span><span style="text-indent: 2em;"> </span></p><p style="text-align: justify; text-indent: 2em;"><br/></p><p style="text-align: justify; text-indent: 2em;">看囊性病變，我們是專業(yè)的！福建省福州肺科醫(yī)院間質(zhì)肺團(tuán)隊(duì)包含呼吸科、影像科、病理科、胸外科和檢驗(yàn)科的專家們，實(shí)力雄厚，經(jīng)驗(yàn)豐富。當(dāng)您看到報(bào)道單上讓人燒腦的“肺囊性病變”、“肺部多發(fā)囊腔樣改變”、“多發(fā)肺氣囊”等描述時，不要焦慮，來“間質(zhì)肺”專病門診，專業(yè)的事交給專業(yè)的人！</p><p style="text-align:center"></p><p style="margin-top: 0; margin-bottom: 0;"><br/></p><p style="margin-top: 0px; margin-bottom: 0px; text-align: center;"><img src="/ueditor/php/upload/image/20240207/1707294218764655.png" width="690" height="245" title="1707294218764655.png" alt="微信圖片_20240207162321.png"/></p><p style="text-align: justify; text-indent: 2em;">間質(zhì)肺專病門診預(yù)約指南：</p><p></p><p style="text-align: justify; text-indent: 2em;">第一步：搜索“福建省福州結(jié)核病防治院”微信公眾號；</p><p style="text-align: justify; text-indent: 2em;">第二步：點(diǎn)擊“服務(wù)”，進(jìn)入“預(yù)約掛號”；</p><p style="text-align: justify; text-indent: 2em;">第三步：選擇“間質(zhì)病門診”。</p><p></p><p style="margin-top: 0; margin-bottom: 0;"><br/></p><p> </p><p> </p><p> </p><p></p><p><br/></p></p> </div> <div id="if7ptrzwxnk" 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