\u56ca\u6027\u80ba\u75c5\u6700\u5e38\u89c1\u7684\u539f\u56e0\u662f\u6dcb\u5df4\u7ba1\u808c\u7624\u75c5(LAM)\u548c\u6717\u683c\u6c49\u65af\u7ec6\u80de\u7ec4\u7ec7\u7ec6\u80de\u589e\u751f\u75c7(LCH)\u3002\u5176\u4ed6\u8f83\u5c11\u89c1\u7684\u56ca\u6027\u80ba\u75be\u75c5\u5305\u62ecBirt-Hogg-Dube\u7efc\u5408\u5f81\u3001\u6dcb\u5df4\u7ec6\u80de\u95f4\u8d28\u6027\u80ba\u708e(LIP)\u548c\u8f7b\u94fe\u6c89\u79ef\u75c5(LCDD)\u3002\u800c\u80ba\u6c14\u80bf\u3001\u8702\u7a9d\u80ba\u3001\u80ba\u7a7a\u6d1e\u548c\u652f\u6c14\u7ba1\u6269\u5f20\u7c7b\u4f3c\u56ca\u6027\u80ba\u75c5\uff0c\u9700\u8981\u8fdb\u884c\u9274\u522b\u8bca\u65ad\u3002<\/p>\n
\u4e00\u3001\u6dcb\u5df4\u7ba1\u808c\u7624\u75c5 (LAM)\uff1a\u662f\u4ee5\u5f25\u6f2b\u6027\u80ba\u56ca\u6027\u75c5\u53d8\u4e3a\u7279\u5f81\u7684\u80bf\u7624\u6027\u75be\u75c5\u3002\u51e0\u4e4e\u53ea\u53d1\u751f\u4e8e\u5973\u6027\u60a3\u8005\u3002\u5e73\u5747\u53d1\u75c5\u5e74\u9f84\u4e3a 40\u5c81\u3002\u4e3b\u8981\u8868\u73b0\u4e3a\u547c\u5438\u56f0\u96be\u3001\u6c14\u80f8\u3001\u4e73\u7cdc\u80f8\u3002LAM \u7684\u786e\u8bca\u4e3b\u8981\u4f9d\u636e\u5178\u578b\u7684\u4e34\u5e8a\u8868\u73b0\u4ee5\u53ca\u6d3b\u68c0\u75c5\u7406\u6216\u8005\u7279\u5f81\u6027\u8868\u73b0, \u5982\u7ed3\u8282\u6027\u786c\u5316\u75c7 (TSC)\u3001\u80be\u8840\u7ba1\u808c\u8102\u7624 (AML)\u3001\u4e73\u7cdc\u80f8\u3001\u4e73\u7cdc\u8179\u7b49\u3002\u8840\u7ba1\u5185\u76ae\u751f\u957f\u56e0\u5b50D (VEGF-D)\u5df2\u4f5c\u4e3a\u65b0 \u7684\u8bca\u65ad\u65b9\u6cd5\u7528\u4e8e\u4e34\u5e8a\u3002\u7ecf\u652f\u6c14\u7ba1\u955c\u80ba\u6d3b\u68c0 (TBLB)\u83b7\u53d6\u80ba\u7ec4\u7ec7\u4e5f\u662f\u65b9\u4fbf\u53ca\u5b89\u5168\u7684\u3002\u897f\u7f57\u83ab\u53f8\u4f5c\u4e3a\u96f7\u5e15\u9709\u7d20\u9776\u86cb\u767d (mTOR)\u6291\u5236\u5242\u662fLAM\u7684\u6709\u6548\u6cbb\u7597\u624b\u6bb5\u3002<\/p>\n<\/p>\n
\u7ed3\u8282\u6027\u786c\u5316\u75c7\u60a3\u8005\uff0c\u4e24\u80ba\u591a\u53d1\u56ca\u6027\u4f4e\u5bc6\u5ea6\u5f71\uff0c\u5f62\u6001\u5448\u5706\u5f62\uff0c\u5448\u5f25\u6f2b\u5206\u5e03\uff0c\u56ca\u95f4\u5939\u6742\u6b63\u5e38\u80ba\u7ec4\u7ec7\uff0c\u808b\u8188\u89d2\u53ef\u7d2f\u53ca\u3002\u53cc\u80be\u591a\u53d1\u8840\u7ba1\u5e73\u6ed1\u808c\u8102\u80aa\u7624\uff0c\u8bca\u65ad\u6dcb\u5df4\u7ba1\u808c\u7624\u75c5\u3002<\/p>\n
\u4e00\u540d\u60a3\u6709\u6dcb\u5df4\u7ba1\u808c\u7624\u75c5\u7684 38 \u5c81\u5973\u6027\u3002a) \u4e0a\u53f6\u548c b) \u4e0b\u53f6\u7684HRCT\u626b\u63cf\u663e\u793a\uff0c\u591a\u4e2a\u8584\u58c1\u56ca\u80bf\u5bf9\u79f0\u5206\u5e03\u4e8e\u53cc\u80ba\uff0c\u4e2d\u95f4\u7684\u80ba\u5b9e\u8d28\u6b63\u5e38\u3002<\/p>\n<\/p>\n<\/p>\n<\/p>\n
\u4e8c\u3001\u6717\u6c49\u6c0f\u7ec6\u80de\u7ec4\u7ec7\u7ec6\u80de\u589e\u591a\u75c7(PLCH):\u662f\u4e00\u79cd\u5438\u70df\u76f8\u5173\u6027\u80ba\u75c5\uff0c\u6700\u5e38\u89c1\u4e8e\u5e74\u8f7b\u4eba\uff0c80%\u5230100%\u7684\u75c5\u4f8b\u62a5\u544a\u6709\u5438\u70df\u53f2,\u80ba\u90e8LCH\u7684\u786e\u5207\u53d1\u75c5\u7387\u548c\u60a3\u75c5\u7387\u5c1a\u4e0d\u6e05\u695a\uff0c\u56e0\u4e3a\u6709\u4e9b\u60a3\u8005\u53ef\u80fd\u65e0\u4e34\u5e8a\u75c7\u72b6\uff0c\u8be5\u75c5\u53ef\u80fd\u662f\u81ea\u53d1\u7684\u3002\u4e34\u5e8a\u4e0a\uff0c\u60a3\u8005\u6700\u5e38\u89c1\u7684\u8868\u73b0\u4e3a\u65e0\u75c7\u72b6\u6027\u54b3\u55fd\u548c\u547c\u5438\u56f0\u96be\uff0c\u4f53\u91cd\u51cf\u8f7b\u3001\u75b2\u52b3\u3001\u80f8\u75db\u548c\u53d1\u70ed\u8f83\u5c11\u3002\u81ea\u53d1\u6027\u6c14\u80f8\u662f\u4e00\u79cd\u516c\u8ba4\u7684\u7279\u5f81\uff0c\u5728\u65e0\u80f8\u819c\u56fa\u5b9a\u7684\u60c5\u51b5\u4e0b\uff0c\u5176\u590d\u53d1\u7387\u8f83\u9ad8\uff0c\u80ba\u6c14\u80f8\u53ef\u80fd\u662f\u80ba\u90e8LCH\u7684\u521d\u671f\u8868\u73b0\uff0cHRCT\u53ef\u4ee5\u53ef\u9760\u5730\u8bca\u65adLCH\u3002\u6700\u80fd\u533a\u5206LCH\u548c\u5176\u4ed6\u80ba\u56ca\u6027\u75be\u75c5\u7684\u7279\u5f81\u662f\u5176\u4e0a\u80ba\u533a\u5360\u4f18\u52bf\uff0c\u808b\u8188\u89d2\u76f8\u5bf9\u6216\u5b8c\u5168\u6b63\u5e38\uff0c\u7ed3\u8282\u7684\u5b58\u5728\u662f\u533a\u5206LAM\u548cLCH\u7684\u4e00\u4e2a\u6709\u5e2e\u52a9\u7684\u7279\u5f81\u3002\u65e9\u671fLCH\u53ef\u80fd\u4e0e\u547c\u5438\u6027\u7ec6\u652f\u6c14\u7ba1\u708e(RB)\u76f8\u4f3c\uff0c\u8868\u73b0\u4e3a\u7ec6\u652f\u6c14\u7ba1\u4e2d\u5fc3\u5c0f\u7ed3\u8282\uff0c\u968f\u7740\u75be\u75c5\u7684\u8fdb\u5c55\uff0c\u56ca\u80bf\u7684\u5f62\u6210\u53d1\u5c55\u3002\u56ca\u80bf\u53ef\u878d\u5408\u5f62\u6210\u4e0d\u89c4\u5219\u7684\u5947\u5f62\u602a\u72b6\uff0c\u8fd9\u662fLCH\u7684\u7279\u5f81\u3002CT\u4e0a\u6240\u89c1\u7684\u5f02\u5e38\u7684\u5e8f\u8d2f\u8fdb\u5c55\u5982\u4e0b:\u7ed3\u8282\u3001\u7a7a\u6d1e\u7ed3\u8282\u3001\u539a\u58c1\u56ca\u80bf\u3001\u8584\u58c1\u56ca\u80bf\u548c\u878d\u5408\u56ca\u80bf\u3002LCH\u7684\u4e34\u5e8a\u548c\u5f71\u50cf\u5b66\u968f\u8bca\uff0c50%\u7684\u60a3\u8005\u75c5\u60c5\u7a33\u5b9a\uff0c25%\u7684\u60a3\u8005\u75c5\u60c5\u81ea\u53d1\u6027\u6d88\u9000\uff0c\u5728\u521d\u6b21\u5373\u6709\u5e7f\u6cdb\u7684\u56ca\u80bf\u6027\u75be\u75c5\uff0c\u9884\u540e\u8f83\u5dee\u3002<\/p>\n<\/p>\n
\u6717\u683c\u6c49\u65af\u7ec6\u80de\u7ec4\u7ec7\u7ec6\u80de\u589e\u751f\u75c7\u3002(A) \u8f74\u4f4d CT \u663e\u793a\u80ba\u5b9e\u8d28\u95f4\u6709\u8bb8\u591a\u5f62\u72b6\u4e0d\u89c4\u5219\u3001\u5947\u5f02\u7684\u56ca\u80bf\u548c\u5c0f\u7ed3\u8282\u3002(B) \u51a0\u72b6\u5207\u9762\u663e\u793a\u4e0a\u80ba\u4e3a\u4e3b\uff0c\u80ba\u5e95\u76f8\u5bf9\u8f83\u5c11\u3002<\/p>\n
27\u5c81\uff0c\u7537\u6027 \u80ba\u90e8\u6717\u683c\u6c49\u6c0f\u7ec6\u80de\u7ec4\u7ec7\u7ec6\u80de\u589e\u591a\u75c7\u9ad8\u5206\u8fa8\u7387CT\u626b\u63cf(a)\u4e0a\u3001b)\u4e2d\u80ba\u533a\u663e\u793a\u53cc\u80ba\u591a\u53d1\u6027\u8584\u58c1\u56ca\u80bf\uff0c\u6709\u4e9b\u56ca\u80bf\u5448\u4e0d\u89c4\u5219\u5f62\u72b6(\u7bad\u5934\u6240\u793a)\u3002\u591a\u53d1\u6027\u5c0f\u7ed3\u8282(\u77ed\u7bad\u5934\u6240\u793a)\u3002<\/p>\n
\uff08A\uff09\u80ba\u5185\u663e\u793a\u591a\u4e2a\u7ed3\u8282\u548c\u56ca\u80bf\uff0c\u7ec4\u7ec7\u5b66\u68c0\u67e5\u663e\u793a\u7ec6\u80de\u7ed3\u8282\uff08B\uff09\uff0c\u5176\u4e2d\u4e00\u4e9b\u6709\u4e2d\u5fc3\u7a7a\u8154\uff08B\uff0c*\uff09\uff0c\u5185\u542b\u8bca\u65ad\u6027\u6717\u683c\u6c49\u65af\u7ec6\u80de\u805a\u96c6\uff0cCD1a \u514d\u75ab\u7ec4\u5316\u67d3\u8272\u9633\u6027\uff08C\uff0c\u68d5\u8272\u67d3\u8272\uff09\uff0c\u662f\u8be5\u75c5\u65e9\u671f\u7ec6\u80de\u9636\u6bb5\u7684\u5178\u578b\u8868\u73b0\u3002\u53e6\u4e00\u540d\u60a3\u8005\u7684\u51a0\u72b6 CT \u56fe\u50cf\u663e\u793a\uff0c\u591a\u53d1\u5947\u5f62\u56ca\u80bf\u4e3b\u8981\u5206\u5e03\u5728\u4e0a\u533a\uff0c\u4f46\u4e0d\u5305\u62ec\u808b\u8188\u89d2\uff0c\u8fd9\u662f\u75be\u75c5\u665a\u671f\u7684\u7279\u5f81\uff08D\uff09\u3002\u665a\u671f\u75be\u75c5\u7684\u5178\u578b\u7ec4\u7ec7\u5b66\u7279\u5f81\u5305\u62ec\u56ca\u6027\u95f4\u9699\uff08E\uff09\uff0c\u4f34\u6709\u767d\u7ec6\u80de\u661f\u72b6\u7ea4\u7ef4\u5316\uff08E\uff0c\u7bad\u5934\uff09\u3002\u5468\u56f4\u5b9e\u8d28\u4e2d\u7ecf\u5e38\u53ef\u89c1\u4e0e\u5438\u70df\u6709\u5173\u7684\u8272\u7d20\u5de8\u566c\u7ec6\u80de\u5806\u79ef\uff08F\uff0c\u7bad\u5934\uff09\u3002\u539f\u59cb\u653e\u5927\u500d\u7387\uff1a2X (E)\uff1b4X(B)\uff1b40X (C)\uff1b1003X (F) \u3002<\/p>\n
PLCH in 22-year-old male smoker. CT shows multiple, irregular, and round, thick- and thin-walled cysts with small irregular nodules (arrows) in both lungs, sparing lung bases and costophrenic angles (not shown), suggestive of PLCH, which was subsequently confirmed histologically.<\/p>\n
\u4e09\u3001\u6dcb\u5df4\u7ec6\u80de\u95f4\u8d28\u6027\u80ba\u708e(LIP)\uff1a\u662f\u4e00\u79cd\u826f\u6027\u6dcb\u5df4\u589e\u751f\u6027\u75be\u75c5\uff0c\u5176\u7ec4\u7ec7\u5b66\u7279\u5f81\u4e3a\u5f25\u6f2b\u6027\u7ec6\u80de\u95f4\u8d28\u6d78\u6da6\uff0c\u7531\u6210\u719f\u7684\u5c0f\u6dcb\u5df4\u7ec6\u80de\u548c\u6d46\u7ec6\u80de\u7ec4\u6210\uff0c\u6d46\u7ec6\u80de\u503e\u5411\u4e8e\u4f4d\u4e8e\u6dcb\u5df4\u5468\u95f4\u8d28\uff0c\u6700\u5e38\u89c1\u7684\u662f\u4e0e\u81ea\u8eab\u514d\u75ab\u7efc\u5408\u5f81\u6216\u514d\u75ab\u7f3a\u9677\uff0c\u5982\u5e72\u71e5\u7efc\u5408\u5f81\u3001\u81ea\u8eab\u514d\u75ab\u6027\u7532\u72b6\u817a\u7efc\u5408\u5f81\u3001\u4eba\u514d\u75ab\u7f3a\u9677\u75c5\u6bd2\u611f\u67d3\u3001Castleman\u75c5\u548c\u6dc0\u7c89\u6837\u53d8\u6027\u7b49\u75be\u75c5\u76f8\u5173\u3002\u80ba\u56ca\u80bf\u89c1\u4e8e\u9ad8\u8fbe80%\u7684\u75c5\u4eba\uff0c\u88ab\u8ba4\u4e3a\u662f\u7531\u4e8e\u652f\u6c14\u7ba1\u5468\u56f4\u7ec6\u80de\u6d78\u6da6\u6240\u81f4\u7684\u6c14\u9053\u963b\u585e\u6240\u81f4\u3002\u4e34\u5e8a\u4e0a\uff0cLIP\u60a3\u8005\u5e38\u89c1\u54b3\u55fd\u3001\u547c\u5438\u56f0\u96be\uff0c\u53ef\u6709\u53d1\u70ed\u3001\u76d7\u6c57\u53ca\u4f53\u91cd\u51cf\u8f7b\u3002\u80ba\u529f\u80fd\u8bd5\u9a8c\u5e38\u6709\u9650\u5236\u578b\u53ca\u4e00\u6c27\u5316\u78b3\u5f25\u6563\u80fd\u529b\u51cf\u4f4e\uff0cLIP\u7684\u4e34\u5e8a\u75c5\u7a0b\u53d8\u5316\u5f88\u5927\u3002\u6709\u4e9b\u75c5\u4eba\u75c5\u60c5\u6539\u5584\u6216\u7a33\u5b9a\u3002\u867d\u7ecf\u6cbb\u7597\uff0c\u4ecd\u6709\u8d85\u8fc7\u4e09\u5206\u4e4b\u4e00\u7684\u75c5\u4eba\u75c5\u60c5\u8fdb\u5c55\u3002HRCT\u4e0a\uff0c\u80ba\u56ca\u80bf\u89c1\u4e8e80%\u7684\u60a3\u8005\uff0c\u5c3d\u7ba1\u6587\u732e\u4e2d\u4e3b\u8981\u63cf\u8ff0\u7684\u662f\u5f25\u6f2b\u6027\u78e8\u73bb\u7483\u5f71\uff0c\u5b9a\u4e49\u4e0d\u6e05\u7684\u5c0f\u53f6\u4e2d\u5fc3\u7ed3\u8282\uff0c\u652f\u6c14\u7ba1\u8840\u7ba1\u548c\u5c0f\u53f6\u95f4\u9694\u589e\u539a\uff0c\u5f25\u6f2b\u6027\u6563\u5728\u56ca\u80bf\u3002\u7136\u800c\uff0c\u5728\u4f5c\u8005\u7684\u4e34\u5e8a\u7ecf\u9a8c\u4e2d\uff0cLIP\u6700\u5e38\u89c1\u7684\u8868\u73b0\u4e3a\u56ca\u6027\u80ba\u75be\u75c5\uff0c\u4ee5\u4e2d\u4e0b\u80ba\u4e3a\u4e3b\uff0c\u56ca\u80bf\u4f4d\u4e8e\u8840\u7ba1\u5468\u56f4\uff0c\u78e8\u73bb\u7483\u5f71\u53ef\u4ee5\u5438\u6536\uff0c\u800c\u56ca\u6027\u6539\u53d8\u662f\u4e0d\u53ef\u9006\u7684\u3002<\/p>\n<\/p>\n
\u6dcb\u5df4\u7ec6\u80de\u95f4\u8d28\u6027\u80ba\u708e(A)\u8f74\u4f4dCT\u663e\u793a\u5927\u91cf\u58c1\u8584\u56ca\u80bf\uff0c\u5176\u4e2d\u4e00\u4e9b\u662f\u8840\u7ba1\u5468\u56f4\u56ca\u80bf\u3002(B)\u80ba\u4e0b\u90e8\u56ca\u80bf\u8f7b\u5ea6\u5360\u4f18\u52bf\u3002(C)LIP\u56ca\u80bf\u7684\u7279\u5f81\u662f\u8840\u7ba1\u5468\u56f4\u56ca\u80bf\uff0c\u8fd9\u53ef\u80fd\u662f\u6709\u5e2e\u52a9\u7684\u7ebf\u7d22.<\/p>\n
\u4e00\u540d 43 \u5c81\u5973\u6027\uff0c\u60a3\u6709\u6dcb\u5df4\u7ec6\u80de\u6027\u95f4\u8d28\u6027\u80ba\u708e\uff0c\u5e76\u4f34\u5e72\u71e5\u7efc\u5408\u5f81\u3002a\uff09\u4e2d\u80ba\u533a\u548c b\uff09\u4e0b\u80ba\u533a\u7684HRCT\u626b\u63cf\u663e\u793a\u53cc\u4fa7\u8584\u58c1\u56ca\u80bf\u3002<\/p>\n
\u56db\u3001Birt-Hogg-Dube (BHD) \u7efc\u5408\u5f81\uff1a\u662f\u4e00\u79cd\u7f55\u89c1\u7684\u5e38\u67d3\u8272\u4f53\u663e\u6027\u9057\u4f20\u75be\u75c5\uff0c\u6700\u521d\u4e8e 1977 \u5e74\u88ab\u63cf\u8ff0\uff0c\u5176\u7279\u5f81\u6027\u76ae\u80a4\u75c5\u53d8\u5305\u62ec\u4e09\u8054\u5f81\uff1a\u7ea4\u7ef4\u8f6f\u9aa8\u7624\u3001\u6bdb\u53d1\u7c98\u819c\u7624\u548c\u76ae\u80a4\u635f\u5bb3\u3002BHD \u7efc\u5408\u5f81\u4e0e\u80be\u810f\u80bf\u7624\uff08\u5305\u62ec\u80bf\u7624\u7ec6\u80de\u7624\u548c\u80be\u7ec6\u80de\u764c\uff09\u3001\u80ba\u56ca\u80bf\u548c\u81ea\u53d1\u6027\u6c14\u80f8\u7684\u5173\u8054\u3002\u7ec4\u7ec7\u75c5\u7406\u5b66\u65e0\u7279\u5f02\u6027\uff1b\u5178\u578b\u7684\u75c5\u7406\u7ed3\u679c\u5305\u62ec\u5145\u6ee1\u7a7a\u6c14\u7684\u95f4\u9699\uff08\u53ef\u80fd\u6709\u8584\u8584\u7684\u7ea4\u7ef4\u58c1\uff09\u548c\u6b63\u5e38\u7684\u80ba\u5b9e\u8d28\u3002BHD \u7efc\u5408\u5f81\u7684\u9884\u540e\u4e3b\u8981\u53d6\u51b3\u4e8e\u5176\u4ed6\u5408\u5e76\u56e0\u7d20\uff0c\u5982\u80be\u7ec6\u80de\u764c\u3002BHD \u7efc\u5408\u5f81\u7684\u56ca\u80bf\u5f80\u5f80\u5927\u5c0f\u4e0d\u4e00\u3001\u5f62\u6001\u5404\u5f02\uff0c\u4e14\u4ee5\u53cc\u4fa7\u548c\u4e0b\u80ba\u533a\u4e3a\u4e3b\u3002\u5f62\u6001\u53d8\u5316\u4ece\u5706\u5f62\u5230\u5375\u5706\u5f62\u548c\u900f\u955c\u72b6\uff0c\u4e00\u4e9b\u8f83\u5927\u7684\u56ca\u80bf\u8868\u73b0\u4e3a\u591a\u9694\u548c\u5206\u53f6\u72b6\u3002\u56ca\u80bf\u7279\u5f81\u6027\u4e3a\u80f8\u819c\u4e0b\uff0c\u5e38\u4e3a\u7eb5\u9694\u65c1\uff0c\u53ef\u6cbf\u80ba\u4e0b\u53f6\u80ba\u52a8\u8109\u548c\u9759\u8109\u7684\u8fd1\u7aef\u5ef6\u4f38\u3002<\/p>\n<\/p>\n
Birt-Hogg-Dube \u7efc\u5408\u5f81\u3002(A\uff09\u80f8\u819c\u4e0b\u56ca\u80bf\u4f4d\u7f6e\u548c\u5206\u53f6\u72b6\uff0c\u6709\u65f6\u5448\u9694\u819c\u72b6\u5f62\u6001\u662f\u5176\u7279\u5f81\u3002(B) \u51a0\u72b6 CT \u663e\u793a\u4ee5\u4e0b\u80ba\u4e3a\u4e3b\u3002(C) \u8179\u90e8\u5bf9\u6bd4\u589e\u5f3a CT \u663e\u793a\u53cc\u4fa7\u80be\u810f\u5f3a\u5316\u80bf\u5757\uff0c\u4e3a\u55dc\u9178\u7ec6\u80de\u7624\uff0c\u5c5e\u4e8e\u826f\u6027\u75c5\u53d8<\/p>\n
Birt-Hogg-Dube \u7efc\u5408\u5f81a) \u80f8\u7247\u663e\u793a\u53cc\u4fa7\u5927\u6ce1\u5f62\u6210\u5e76\u4f34\u6709\u5de6\u4fa7\u57fa\u5e95\u6c14\u80f8\u3002b, c) \u4e00\u5468\u540e\u8fdb\u884c\u7684\u9ad8\u5206\u8fa8\u7387\u8ba1\u7b97\u673a\u65ad\u5c42\u626b\u63cf\u663e\u793a\u53cc\u80ba\u5185\u6709\u5927\u5c0f\u4e0d\u7b49\u7684\u8584\u58c1\u56ca\u80bf\uff0c\u6709\u4e9b\u5448\u900f\u955c\u72b6\uff0c\u5e76\u6709\u7279\u5f81\u6027\u7684\u4e0b\u80ba\u533a\u5206\u5e03\u3002\u5de6\u80ba\u8fd8\u6b8b\u7559\u4e00\u4e2a\u5c0f\u6c14\u80f8\uff08\u7bad\u5934\uff09\u3002<\/p>\n
Birt-Hogg-Dub\u00e9 syndrome with recurrent pneumothorax in 47-year-old woman (A) and 22-year-old woman (B) who were mother and daughter. A. CT shows multiple, variable-sized, thin-walled cysts with lower subpleural lung predominance. Chest drainage tube (arrow head) is noted in right hemithorax for pneumothorax. B. CT shows multiple thin-walled cysts of various sizes and shapes in both lungs, with left lower lung dominance, and pneumothorax in left hemithorax.<\/p>\n
\u4e94\u3001Light Chain Deposition Disease\uff08LCDD)\u8f7b\u94fe\u6c89\u79ef\u75c5:\u662f\u4e00\u79cd\u7f55\u89c1\u75be\u75c5\uff0c\u5176\u7279\u5f81\u662f\u975e\u7ea4\u7ef4\u72b6\u3001\u65e0\u5b9a\u5f62\u7269\u8d28\u7684\u6c89\u79ef\uff0c\u8fd9\u79cd\u7269\u8d28\u4e0d\u50cf\u6dc0\u7c89\u6837\u53d8\u6027\u90a3\u6837\u5177\u6709 b \u578b\u7ed3\u6784\uff0c\u4e5f\u4e0d\u4e0e\u521a\u679c\u7ea2\u7ed3\u5408\u3002LCDD\u662f\u4e00\u79cd\u591a\u5668\u5b98\u75be\u75c5\uff0c\u5e38\u7d2f\u53ca\u80be\u810f\u3002\u5fc3\u810f\u548c\u809d\u810f\u662f\u4e0b\u4e00\u4e2a\u6700\u5e38\u89c1\u7684\u53d7\u7d2f\u5668\u5b98\uff0c\u80ba\u90e8\u53d7\u7d2f\u7f55\u89c1\uff0c\u901a\u5e38\u65e0\u4e34\u5e8a\u75c7\u72b6\uff0c\u4e2d\u5e74\u7537\u6027\u662f\u6700\u5e38\u89c1\u7684\u60a3\u75c5\u4eba\u7fa4\uff0c\u5176\u4e2d 75% \u7684\u4eba\u60a3\u6709\u6f5c\u5728\u7684\u6d46\u7ec6\u80de\u5f02\u5e38\uff0c\u5982\u591a\u53d1\u6027\u9aa8\u9ad3\u7624\u6216\u74e6\u5c14\u767b\u65af\u7279\u7f57\u59c6\u5de8\u7403\u86cb\u767d\u8840\u75c7\u3002HRCT\u8868\u73b0\u4e3a\u591a\u53d1\u6027\u3001\u53cc\u4fa7\u6216\u5b64\u7acb\u6027\u7ed3\u8282\uff0c\u5927\u5c0f2\uff5e5mm\u4e0d\u7b49\u3002LCDD\u4e3b\u8981\u8868\u73b0\u4e3a\u56ca\u6027\u80ba\u75be\u75c5\uff0c\u4f34\u6709\u963b\u585e\u6027\u6162\u6027\u80ba\u8870\u7aed\uff0c\u6700\u7ec8\u9700\u8981\u80ba\u79fb\u690d\u3002\u968f\u8bbf\u80ba\u56ca\u6027\u75be\u75c5\u53ef\u4ee5\u8fdb\u5c55\uff0c\u4f34\u6709\u7ed3\u8282\u548c\u5b9e\u53d8\u3002\u6dc0\u7c89\u6837\u53d8\u4e5f\u53ef\u80fd\u51fa\u73b0\u7c7b\u4f3c\u7684\u56ca\u80bf\uff0c\u5176\u5e38\u5e38\u4f34\u6709\u9499\u5316\u7ed3\u8282<\/p>\n<\/p>\n
\u8f7b\u94fe\u6c89\u79ef\u75c5:\u5de6\u80ba\u8840\u7ba1\u5468\u56f4\u56ca\u80bf\u662f\u591a\u53d1\u6027\u9aa8\u9ad3\u7624\u60a3\u8005\u8f7b\u94fe\u6c89\u79ef\u75c5\u7684\u7279\u5f81\u3002<\/p>\n
\u516d\u3001\u5176\u4ed6\u56ca\u6027\u80ba\u75c5\uff1a\u5b64\u7acb\u6027\u80ba\u56ca\u80bf\u5e38\u89c1\u4e8e\u5176\u4ed6\u6b63\u5e38\u7684\u4e2a\u4f53\u3002\u5176\u4e2d\u6709\u4e9b\u53ef\u80fd\u662f\u4ee5\u524d\u611f\u67d3\u6216\u6c14\u538b\u5916\u4f24\u7684\u6b8b\u7559\u7269\u3002\u5148\u5929\u6027\u80ba\u56ca\u80bf\u6216\u5148\u5929\u6027\u80ba\u6c14\u9053\u56ca\u6027\u7578\u5f62\u901a\u5e38\u662f\u5b64\u7acb\u7684\u3002\u56ca\u80bf\u4e5f\u53ef\u53d1\u751f\u4e8e\u5176\u4ed6\u5f25\u6f2b\u6027\u80ba\u90e8\u75be\u75c5\uff0c\u5305\u62ec\u5361\u6c0f\u80ba\u56ca\u866b\u80ba\u708e\u3001\u56ca\u6027\u80ba\u8f6c\u79fb\u548c\u8131\u843d\u6027\u95f4\u8d28\u6027\u80ba\u708e(DIP)\u3002\u6240\u6709\u8fd9\u4e9b\u75be\u75c5\u90fd\u6709\u76f8\u5173\u7684\u4e34\u5e8a\u548c\u5f71\u50cf\u5b66\u7279\u5f81\uff0c\u8fd9\u4e9b\u7279\u5f81\u5e94\u8be5\u5bfc\u81f4\u6b63\u786e\u7684\u8bca\u65ad\u3002<\/p>\n
1.PJP\u5361\u6c0f\u80ba\u56ca\u866b\u80ba\u708e:\u5e38\u89c1\u4e8eHIV\u6216\u514d\u75ab\u7f3a\u9677\u60a3\u8005\uff0c\u4e24\u80ba\u4e0a\u533aCT\u663e\u793a\u53cc\u4fa7\u5f25\u6f2b\u6027GGO\u80cc\u666f\u4e0b\u7684\u591a\u53d1\u4e0d\u89c4\u5219\u7684\u539a\u58c1\u548c\u8584\u58c1\u56ca\u80bf\u3002<\/p>\n<\/p>\n
\u4e00\u540d\u60a3\u6709\u80ba\u5b62\u5b50\u866b\u80ba\u708e\u7684 37 \u5c81\u7537\u5b50\u3002a) \u4e0a\u53f6\u548c b) \u4e0b\u53f6\u7684HRCT\u626b\u63cf\u663e\u793a\uff0c\u8584\u58c1\u56ca\u80bf\u4f34\u6709\u78e8\u73bb\u7483\u4e0d\u900f\u660e\uff0c\u5206\u5e03\u5728\u6574\u4e2a\u80ba\u90e8\u3002<\/p>\n
2.\u80ba\u6c14\u80bf\uff1a\u662f\u80ba\u5b9e\u8d28\u7684\u7834\u574f\uff0c\u4e0e\u80ba\u7684\u89e3\u5256\u7ed3\u6784\u6709\u5173\uff0c\u53ef\u5206\u4e3a\u5c0f\u53f6\u4e2d\u5fc3\u578b\u3001\u9694\u5468\u578b\u548c\u5c0f\u53f6\u578b\uff0c\u6ca1\u6709\u8584\u800c\u53ef\u611f\u77e5\u7684\u56ca\u58c1\uff0c\u4e2d\u5fc3\u53ef\u89c1\u8840\u7ba1\u5f71\uff0c\u8fd9\u662f\u533a\u5206\u80ba\u6c14\u80bf\u548c\u771f\u6b63\u80ba\u56ca\u80bf\u7684\u7279\u5f81\u3002<\/p>\n<\/p>\n
\u80ba\u6c14\u80bf\u3002(A\uff0cB\uff09\u4e24\u540d\u60a3\u8005\u7684\u8f74\u4f4d CT \u663e\u793a\u4e0a\u80ba\u6709\u51e0\u4e2a\u5706\u5f62\u7684\u900f\u660e\u533a\u57df\uff0c\u4ee3\u8868\u8fd9\u4e9b\u6709\u957f\u671f\u5438\u70df\u53f2\u7684\u60a3\u8005\u7684\u4e2d\u5fc3\u53f6\u80ba\u6c14\u80bf\u3002<\/p>\n
3.\u8702\u7a9d\uff1a\u4ece\u75c5\u7406\u5b66\u89d2\u5ea6\u6765\u770b\uff0c\u8702\u7a9d\u7ec4\u7ec7\u4ee3\u8868\u4e86\u80ba\u5b9e\u8d28\u7684\u7834\u574f\u548c\u7ea4\u7ef4\u5316\uff0c\u5f62\u6210\u56ca\u6027\u95f4\u9699\uff0c\u4ee3\u8868\u4e86\u6269\u5f20\u7684\u547c\u5438\u7ec6\u652f\u6c14\u7ba1<\/p>\n<\/p>\n
4.\u7a7a\u6d1e\uff1a\u80ba\u56ca\u80bf\u4e0e\u7a7a\u6d1e\u7684\u533a\u522b\u4e3b\u8981\u53d6\u51b3\u4e8e\u80ba\u58c1\u7684\u539a\u5ea6\uff0c\u754c\u9650\u4e3a>4mm<\/p>\n<\/p>\n
\u7a7a\u6d1e\uff0c\u8089\u82bd\u80bf\u6027\u611f\u67d3\u7684\u75c5\u7076<\/p>\n
5.\u652f\u6c14\u7ba1\u6269\u5f20\uff1a\u652f\u6c14\u7ba1\u6269\u5f20\u662f\u6307\u6c14\u9053\u4e0d\u53ef\u9006\u7684\u6269\u5f20\uff0c\u53ef\u7531\u591a\u79cd\u539f\u56e0\u5f15\u8d77\u3002\u6709\u4e09\u79cd\u4e3b\u8981\u7c7b\u578b(\u4f9d\u5176\u4e25\u91cd\u7a0b\u5ea6\u800c\u5b9a):\u5706\u67f1\u578b\u3001\u7c7b\u9759\u8109\u66f2\u5f20\u548c\u56ca\u6027\u3002\u56ca\u6027\u652f\u6c14\u7ba1\u6269\u5f20\u662f\u6700\u5e38\u89c1\u7684\u7c7b\u578b\uff0c\u4e0e\u80ba\u56ca\u80bf\u76f8\u4f3c\uff0c\u4f46\u53ef\u901a\u8fc7\u8ffd\u8e2a\u5176\u4e0e\u6c14\u9053\u7684\u76f8\u90bb\u5173\u7cfb\u6613\u4e8e\u9274\u522b\uff0c\u8fd9\u5728\u591a\u5e73\u9762\u91cd\u5efa\u65f6\u53ef\u80fd\u66f4\u51c6\u786e\u3002<\/p>\n<\/p>\n
\u652f\u6c14\u7ba1\u6269\u5f20\u3002(A\uff09\u8f74\u4f4d CT \u663e\u793a\u80ba\u5185\u6709\u5706\u5f62\u900f\u660e\u533a\uff0c\u58c1\u8584\u3002(B\uff09\u7136\u800c\uff0c\u51a0\u72b6\u4f4d\u91cd\u5851\u663e\u793a\u8fd9\u4e9b\u900f\u660e\u533a\u4e0e\u6c14\u9053\u6bd7\u8fde\u3002<\/p>\n
6.\u8131\u843d\u6027\u95f4\u8d28\u6027\u80ba\u708e(DIP)\uff1a\u5728\u78e8\u73bb\u7483\u5f71\u4e2d\u4f34\u6709\u5c0f\u56ca\u80bf\uff0c\u4e3b\u8981\u4f4d\u4e8e\u4e0b\u80ba\uff0c\u5206\u5e03\u51e0\u4e4e\u5bf9\u79f0\u3002<\/p>\n<\/p>\n
7.\u56ca\u6027\u80ba\u8f6c\u79fb\uff1a<\/p>\n<\/p>\n
Fig. 10. Cystic metastasis from scalp angiosarcoma with recurrent pneumothorax in 71-year-old man. A. CT shows multiple cystic or cavitary nodules (arrow) that were histologically confirmed as metastasis. Pneumothorax is noted with drainage tube (arrow head). B. CT performed after 2 months shows variable-sized thin-walled cystic lesions in both lungs that greatly increased in size and number as compared with previous CT scan (A).<\/p>\n
8.\u652f\u6c14\u7ba1\u80ba\u56ca\u80bf\uff1a\u652f\u6c14\u7ba1\u80ba\u56ca\u80bf\u662f\u4e00\u79cd\u53d1\u80b2\u5f02\u5e38\uff0c\u80ce\u513f\u671f\u652f\u6c14\u7ba1\u51fa\u73b0\u5f02\u5e38\u7684\u840c\u82bd\u6216\u5206\u652f\u7f3a\u9677\u3002<\/p>\n<\/p>\n
9.\u5148\u5929\u6027\u80ba\u6c14\u9053\u7578\u5f62(CPAM):\u662f\u4e00\u79cd\u7531\u5f02\u5e38\u652f\u6c14\u7ba1\u7ed3\u6784\u589e\u751f\u5f15\u8d77\u7684\u56ca\u6027\u548c\u975e\u56ca\u6027\u80ba\u75c5\u53d8<\/p>\n<\/p>\n
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