【摘要】 【目的】探讨中国人肺淋巴管肌瘤病(LAM)的临床表现、肺功能、影像学特点、病理改变及疗效和预后。【方法】通过中国生物医学文献光盘数据库(CBM-Disk)检索1986年1月至2005年6月有关报道LAM的文献,剔除重复累积报道病例,结合本院收治的3例,回顾性分析中国人肺LAM患者的临床资料。【结果】总共报道了26例中国人肺LAM,均经过病理确诊。女24例(92%),男2例(8%),发病平均年龄35岁(中位数:36岁,范围:5~69岁),其中21例(81%)为中青年生育期女性;合并腹膜后LAM 5例(19%),颅内结节性硬化3例(12%),结节性硬化2例(8%),肾脏血管肌脂瘤2例(8%),子宫肌瘤2例(8%),肺腺癌1例(4%);死亡9例(35%),均为呼吸衰竭,平均病程7.5年(中位数4.5年,范围半年至23年);主要临床表现为呼吸困难22例(85%)、咳嗽14例(54%)、咳痰和咯血13例(50%)、气胸13例(50%)、乳糜胸10例(38%)、胸痛5例(19%)、月经失调3例(12%)、腹痛2例(8%)、乳糜腹1例(4%);肺功能检查18例,其中弥散性肺功能障碍16例(89%),阻塞性肺功能障碍11例(61%),限制性肺功能障碍10例(56%);胸部影像学表现:胸片网状改变18例(69%)、小结节状或小囊阴影8例(31%)、蜂窝阴影5例(19%),高分辨CT(HRCT)扫描26例均表现弥漫性薄壁囊状阴影;抗雌激素治疗16例,均有一定短期效果。【结论】与世界其他国家一样,中国人LAM主要侵袭生育期妇女,主要临床表现为呼吸困难、咳嗽、咳痰和咯血,其次为气胸、乳糜胸;即使进行了抗雌激素治疗,病情依然进行性加重。更多还原

【Abstract】 【Objective】To study the clinical features,pulmonary function tests,radiological findings,pathological changes,treatment and prognosis of pulmonary lymphangioleiomyomatosis(PLAM) in China.【Methods】Chinese Biomedical Disk(CBM-Disk) was researched to identify Chinese LAM from its starting date to the first-half year of 2005 and repeatedly reported cases excluded,a retrospective analysis of all cases of Chinese LAM with our reported 3 cases was made.【Results】Twenty six patients were confirmed pathologically.Among 26 patients,24 were female(92%) and 2 were male(8%).The mean age at onset of symptoms was 35(5~69) years old.Twenty one patients(81%) were women of childbearing age.Extrapulmonary presentations included retroperitoneal LAM(5 cases,19%),cerebral tuberous sclerosis(3 cases,12%),tuberous sclerosis(2 cases,8%),renal angiomyolipomas(2 cases,8%) and hysteromyoma(2 cases,8%).The complication had lung adenocarcinoma(1 case,4%).Nine(35%) died of respiratory failure.The mean duration of disease since the onset of symptoms was 7.5(0.5~23) years.The main clinical manifestations included dyspnea(22 cases,85%),cough(14 cases,54%),hemoptysis(13 cases,50%),pneumothorax(13 cases,50%),chylothorax(10 cases,38%), chest pain(5 cases,19%),irregular menstruation(3 cases,12%) and abdominal pain(2 cases,8%).Eighteen patients took pulmonary function test with the results of obstructive ventilation disturbance in 11 cases(61%) and diffusive ventilation disturbance in 16 cases(89%).Chest X-ray findings showed reticular changes(18 cases,69%),nodular infiltration or small cyst changes(8 cases,31%) and honeycomb changes(5 cases,19%).All the cases had characteristic thin-walled cysts throughout the whole lung zones on high resolution computed tomographic(HRCT) scanning.Sixteen patients were treated with progesterone,its effect was short.【Conclusion】As in other countries,in China LAM occurs commonly in women and progresses to dyspnea,hemoytysis,pneumothorax and chylothorax despite hormonal treatment.更多还原