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Tuberous sclerosis associated lymphangioleiomyomatosis: a case report
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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.
Keywords: Tuberous sclerosis, (Lymphangioleiomyomatosis), Cysticlungdisease, (Pneumothorax),Genetics.
Pakistan Medical Association
Title: Tuberous sclerosis associated lymphangioleiomyomatosis: a case report
Description:
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.
Keywords: Tuberous sclerosis, (Lymphangioleiomyomatosis), Cysticlungdisease, (Pneumothorax),Genetics.
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