结节性硬化
淋巴管平滑肌瘤病
医学
疾病
介绍(产科)
发病机制
血管平滑肌脂肪瘤
罕见病
病理
气胸
皮肤病科
儿科
内科学
外科
肾
作者
Davide Elia,Roberto Cassandro,Antonella Caminati,Francesca Luisi,Sergio Harari
出处
期刊:Presse Medicale
[Elsevier]
日期:2023-09-01
卷期号:52 (3): 104173-104173
被引量:2
标识
DOI:10.1016/j.lpm.2023.104173
摘要
Lymphangioleiomyomatosis (LAM) is an ultra-rare, slowly progressive neoplastic cystic disease, belonging to the group of PEComas. It can occur sporadically or associated to tuberous sclerosis complex disease and affects mainly women in child-birth age. Dyspnoea is the most frequent symptom referred to the time of diagnosis, however spontaneous pneumothorax may be a typical presentation associated to extrathoracic manifestations, such as renal angiomyolipomas. In the last decade, important advances in understanding molecular mechanisms underlying the LAM pathogenesis have been reached. It has allowed to obtain improvements in the research of novel biomarkers, treatment and a better management of the disease.
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