Gao Peng, Huang Rong, Cai Bai-qiang, Liu Hong-rui
Department of Respiratory Disease, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2004 Jun;26(3):306-9.
To improve the diagnosis and treatment of lymphangioleiomyomatosis (LAM).
The clinical data of four cases of lymphangioleiomyomatosis diagnosed in our hospital were analyzed and corresponding literature was reviewed.
Lymphangioleiomyomatosis, a rare disorder of unknown cause that occured almost exclusively in women of child bearing ages, was characterized microscopically by atypical smooth muscle proliferation. It could occur as a sporadic diseases or as a part of tuberous sclerosis complex (TSC). LAM was associated with progressive dyspnea, recurrent spontaneous pneumothoraces, chylous effusions, and hamartomas.
Clinicians should be familiar with the characteristics of LAM so that diagnosis and treatment can be made earlier to improve the prognosis.
提高淋巴管平滑肌瘤病（LAM）的诊断与治疗水平。
分析我院诊断的4例淋巴管平滑肌瘤病的临床资料，并复习相关文献。
淋巴管平滑肌瘤病是一种病因不明的罕见疾病，几乎仅发生于育龄女性，镜下特征为非典型平滑肌增生。它可作为散发性疾病出现，也可作为结节性硬化症（TSC）的一部分。LAM与进行性呼吸困难、反复自发性气胸、乳糜胸及错构瘤有关。
临床医生应熟悉LAM的特点，以便尽早作出诊断与治疗，改善预后。
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