新医学2016，Vol.47Issue(6)：408-411,4.DOI:10.3969/j.issn.0253-9802.2016.06.015
肺淋巴管平滑肌瘤病一例
Pulmonary lymphangioleiomyomatosis:a case report
摘要
Abstract
Pulmonary lymphangioleiomyomatosis (PLAM)is a rare diffuse pulmonary disease,fre-quently occurring in women of reproductive age.PLAMis clinically characterized as dyspnea,cough,hemopty-sis and pneumothorax.Pulmonary function test reveals obstructive ventilatory disturbance in PLAM patients with poor clinical prognosis.In this article,a 42-year-old woman diagnosed with PLAM was reported.She presented with the clinical manifestations mainly including shortness of breath,dyspnea and hemoptysis,ac-companied with hypoxemia and pulmonary diffusion function impairment.High-resolution pulmonary computer-ized tomography revealed thin-walled air-filled cysts were diffusively distributed throughout bilateral lungs.The diagnosis of PLAMwas validated by transbronchoscopy biopsy.Clinical symptoms were alleviated after 8-month of oral administration of progestogen.However,no improvement was obtained in terms of the pulmonary le-sions.The patient was being followed up and remained physically stable.关键词
肺淋巴管平滑肌瘤病/呼吸困难/平滑肌增生Key words
Pulmonary lymphangioleiomyomatosis/Dyspnea/Proliferation of smooth muscle引用本文复制引用
邹小玲,陈壮桂,冯定云,周宇麒..肺淋巴管平滑肌瘤病一例[J].新医学,2016,47(6):408-411,4.基金项目
国家自然科学基金资助项目（81470219）；广东省科技计划项目基金资助项目 （）
