摘要
Abstract
Objective To summarize the clinical and pathological features,treatment progress and prognosis of pulmonary lymphangioleiomyomatosis ( PLAM) in Chinese population in the last thirty years. Methods The study used〝 pulmonary lymphangioleiomyomatosis〝 and 〝 PLAM〝 as key words,searched China Hospital Knowledge Database(CHKD) and China Biology Medicine database( CBM),and analysed related data from Chinese literatures which were published from January 1979 to October 2013. Results 91 articles were included in this study,among 130 patients,1(0. 8% )was man and 129(99. 2% )were women. Dyspnea was the most common clinical manifestation(94. 6% ,123 / 130),followed by cough, pneumothorax,chest pain,expectoration,chest tightness,haemoptysis and chylothorax. 122 cases were diagnosed by lung biopsy,the most common lung biopsy method was bronchoscopic biopsy(41. 0% ,50 / 122),followed by thoracoscopic biopsy or pleural biopsy(26. 2% ,32 / 122). The most common extrapulmonary manifestation or complication was retroperitoneal lymph node involvement(52. 1% ,25 / 48),followed by renal angiomyolipoma(12. 5% ,6 / 48)and chyloperitoneum(12. 5% ,6 /48). The most common chest imaging manifestation was multiple different sized thin - walled cystic shadows in whole lung (93. 8% ,122 / 130),followed by pneumothorax( 50. 0% ,65 / 130 )and pleural effusion( 40. 8% ,53 / 130 ). 86 cases received pulmonary function test,the most common form of pulmonary dysfunction was obstructive ventilation dysfunction (65. 1% ,56 / 86)and diffusion dysfunction(38. 4% ,33 / 86). Pathological findings:abnormal smooth muscle cells clustered round alveolar wall,lymphatic vessel and blood vessel,cystic nodules came into being. 9 cases had lung transplant surgery,all the treatments were effective. Conclusion In China,PLAM occurs commonly in child - bearing age women,the most commom manifestation is progressive dyspnea,the imaging and pathological manifestation is characteristic,there is no effective medical therapy,lung transplant surgery is safe and effective,so it is the preferred treatment for PLAM patients.关键词
淋巴管肌瘤病/肺/文献复习Key words
Lymphangioleiomyomatosis/Lung/Literature review分类
医药卫生
