诊断学理论与实践2023，Vol.22Issue(5)：501-506,6.DOI:10.16150/j.1671-2870.2023.05.013
淋巴管平滑肌瘤病及肺部影像诊断研究进展
Pulmonary imaging manifestations and related research progress of lymphangioleiomyomatosis
摘要
Abstract
Lymphangioleiomyomatosis(LAM)is a rare multisystem neoplastic disease and is primarily affected women of childbearing age and premenopausal women.LAM lesions involve the lungs[known as pulmonary Lymphangi-oleiomyomatosis(PLAM)],kidneys[such as angiomyolipoma(AML)],and the lymphatic system(including lymphangioleio-myomas and chylous effusions).As the disease progresses,LAM disrupts lung tissue,alters lung structure,and leads to the development of lymphangioleiomyomas in the chest and abdominal lymphatic ducts.Early symptoms in LAM patients are mild,and clinical presentations lack specificity,making misdiagnosis common.Death can occur due to pulmonary function deterioration and recurrent pneumothorax.Currently,lung transplantation is considered the only effective treatment,al-though recurrence rates are relatively high.High-resolution computer tomography(HRCT)of the chest is a key diagnostic tool for LAM,which aid not only in the diagnosis but also in assessing the severity and prognosis of the condition.With the rapid development of medical imaging technology,particularly the use of photon counting detector CT(PCD-CT),which of-fers high resolution and noise reduction capabilities,significant improvements in image quality can be achieved.Compared to traditional CT scans,PCD-CT reduces radiation exposure by 35.7%,making it highly suitable for diagnosing and long-term monitoring of LAM.关键词
淋巴管平滑肌瘤病/影像学/光子计数探测器计算断层摄影Key words
Lymphangio-leiomyomatosis/Radiology/Photon counting detector computed tomography分类
医药卫生引用本文复制引用
董莱,王炜,吴家良,刘嬿溥,管鑫,陈克敏..淋巴管平滑肌瘤病及肺部影像诊断研究进展[J].诊断学理论与实践,2023,22(5):501-506,6.基金项目
上海市进一步加快中医药传承创新发展三年行动计划ZY(2021-2023)-0207-02 （2021-2023）
