结节性硬化
淋巴管平滑肌瘤病
医学
TSC2
病理
气胸
肺
血管平滑肌脂肪瘤
皮肤病科
肾
内科学
放射科
细胞凋亡
生物化学
化学
PI3K/AKT/mTOR通路
作者
Aiman Mushir,Sohail Akhtar,Mujahid Hussain
摘要
Lymphangioleiomyomatosis (LAM) is a multisystem disorder that primarily affects the lung. Tuberous sclerosis complex (TSC) is characterized by multiple benign tumours of the skin, brain, eyes, heart, lung, liver, and kidney. LAM can be either sporadic (sporadic-LAM) or in association with Tuberous Sclerosis (TSC-LAM). Many clinical, radiologic, and pathologic features are shared between TSC and sporadic variants. We present a case admitted at The Indus Hospital Karachi with pneumothorax and multiple manifestations of TSC-LAM.
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