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2019年, 第13卷 第2期
Lymphangioleiomyomatosis (LAM) is a rare chronic and progressive cystic lung disease in women. Typically, on CT scan of the chest, diffuse thin-walled loofah sac-like cysts are shown. Slowly progressive dyspnea, pneumothorax, chylothorax, or renal angiomyolipomas are the main manifestations of LAM. LAM can be sporadic or associated with tuberous sclerosis complex. Pulmonary hypertension is a major complication in advanced stage of LAM, indicating poor prognosis. Sirolimus, an effective treatment for LAM, may potentially alleviate LAM-related pulmonary hypertension. (Image created by Keqi Chen, Department of Respiratory Medicine, Peking Union Medical College Hospital. Courtesy of Dr. Kaifeng Xu. See pages 259-266 by Xiuxiu Wu et al. for more information.)[展开] ...