“ This is a rare interstitial lung disease that is more common in non-smoking women of child-bearing age. Symptoms worsen in pregnancy or w...
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This is a rare interstitial lung disease that is more common in non-smoking women of child-bearing age. Symptoms worsen in pregnancy or with smoking. May present with spontaneous pneumothorax. Associated with tuberous sclerosis (up to 40% of tuberous scleroses).
PLAIN FILM
• Normal initially, then hyper-expansion and small cysts.
• Check for a pneumothorax.
HRCT
• Uniform, thin-walled cysts with normal intervening lung.
• Chylous pleural effusions.
• Pneumothorax.
• Nodules are unusual.
• HRCT commonly abnormal in affected children with tuberous sclerosis.
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