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The literature suggests that 10% to 20% of adult patients with neurofibromatosis have associated interstitial lung disease. Characteristics of such involvement, as present in the case reported herein, include bilateral lower lobe fibrosis and may include bullous and cystic changes in advanced cases. In addition to pulmonary fibrosis, neurofibromatosis may have other intrathoracic associations; including "dumbbell" neurofibromas, intercostal neurofibromas, and intrathoracic meningoceles.
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Steven A. Davis (1978) studied this question.
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