Objective To summarize the clinical features of lymphangioleiomyomatosis ( LAM) in Chinese population. Methods The first case of LAM in China was reported in 1981. All cases of LAM reported in Chinese literature from January 1981 to December 2009 were reviewed. The cases for analysis were included with a diagnosis of LAM confirmed or probable according to the guidelines of European Respiratory Society, and with individual clinical data available. Results A total of 111 cases of LAM werepooled for analysis. All cases were female. The most frequent onset symptomof LAMwas dyspnea ( 51. 4% ) .The main clinical manifestations included dyspnea ( 93. 4% ) , pneumothorax ( 48. 6% ) , cough ( 45. 7% ) ,hemoptysis ( 35. 1% ) , and chylothorax ( 33. 3% ) . 11 cases were associated with tuberous sclerosis complex.An obstructive with or without restrictive abnormality in pulmonary function testing was observed in 60 /67 cases. Diffusion impairment was also common ( 45 /46) . Of the 109 patients, computerized tomography of chest showed thin-walled air-filled cysts throughout both lungs in 104 cases, multiple bullae in 3 cases, andhoneycomb changes in 2 cases. 102 cases were diagnosed based on pathological findings. Misdiagnosis was found in 44 cases, the median time of misdiagnosis was 24 months. Conclusion Awareness of LAMshould be raised when an adult female who presents with an unexplained dyspnea, especially combined with repeatedpneumothorax or chylothorax.
Objective To evaluate the diagnostic value of vascular endothelial growth factor (VEGF)-D detection for the diagnosis of lymphangioleiomyomatosis (LAM) by Meta-analysis. Methods Literatures published before August 2017 were retrieved from PubMed, Embase, China Biology Medicine database, China National Knowledge Internet, Wangfang, and VIP database to retrieve the study about VEGF-D detection for LAM. The studies were screened according to the inclusive and exclusive criteria, the data were extracted, the quality was assessed and the Meta-analysis was performed with related statistical software. Results Six primary studies were included and 521 patients met the inclusion criterion. The Meta-analysis showed the pooled sensitivity, specificity, positive likelihood ratio, negative likelihood ratio and diagnostic odds ratio of 0.75 [95% confidence interval (CI) (0.70, 0.79)], 0.95 [95%CI (0.91, 0.98)], 16.20 [95%CI (8.70, 30.19)], 0.20 [95%CI (0.10, 0.40)] and 89.49 [95%CI (38.46, 208.22)], respectively. The area under the curve was 0.953 9. Conclusions VEGF-D detection showes a good diagnostic value for LAM. A positive result is more clinical meaningful compared with a negative result, helping for the confirmation of the disease.