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Year : 2018  |  Volume : 6  |  Issue : 3  |  Page : 25-28

Neurofibromatosis associated lung disease

1 Professor, Dept of General Medicine, MVJ, MC & RH
2 Post graduate, Dept of General Medicine, MVJ, MC & RH

Correspondence Address:
R Venkateswara Reddy
Department of General Medicine, MVJ, MC & RH

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Source of Support: None, Conflict of Interest: None

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Von Recklinghausen disease or neurofibromatosis type 1 is an autosomal dominant dysplasia of ectoderm and mesoderm with a variable clinical expression characterized by neuro fibromas, café-au-lait spots and hamartomas of iris (Lisch nodules). Neurofibromatosis has a prevalence of 1 in 3000 and in 30-50% of cases there is no family history of the disease1. The involvement of respiratory system in these patients is very rare. We present an interesting case of a patient with Neurofibromatosis induced lung disease in the form of multiple thin walled bullae, fibrosis and bronchiectasis.

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