Department of Pulmonology, The Indus Hospital, Karachi, Pakistan.
J Pak Med Assoc. 2023 Apr;73(4):908-911. doi: 10.47391/JPMA.5546.
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.
淋巴管平滑肌瘤病（LAM）是一种多系统疾病，主要影响肺部。结节性硬化症复合征（TSC）的特征是皮肤、脑、眼、心脏、肺、肝和肾的多个良性肿瘤。LAM 可以是散发性的（散发性-LAM），也可以与结节性硬化症（TSC-LAM）有关。TSC 和散发性变异之间有许多临床、放射学和病理学特征是共有的。我们在卡拉奇的 Indus 医院介绍了一例气胸和 TSC-LAM 多种表现的病例。
