结节性硬化症相关性淋巴管平滑肌瘤病

定义

这是一种罕见的肺部疾病，导致整个肺部、支气管、肺泡间隔、血管周围间隙和淋巴管内无序平滑肌增生（平滑肌瘤），造成小气道阻塞（导致肺囊肿形成和气胸）和淋巴管阻塞（导致乳糜性胸水）。这一诊断与一种罕见的多系统遗传病有关，它导致非恶性肿瘤在大脑和其他重要器官（如肾脏、心脏、眼睛、肺和皮肤）生长。

This is a rare lung disease that results in a proliferation of disorderly smooth muscle growth (leiomyoma) throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). This diagnosis is associated with a rare multi-system genetic disease that causes non-malignant tumours to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin.

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• 结节性硬化症相关性淋巴管平滑肌瘤病