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Year : 2021  |  Volume : 10  |  Issue : 1  |  Page : 132-135

Pulmonary neurofibromatosis with bilateral bronchiectasis: A rare thoracic manifestation

1 Department of Respiratory Medicine, Ram Manohar Lohiya Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Respiratory Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India
3 Department of Pathology, T. S. M Medical College and Hospital, Lucknow, Uttar Pradesh, India

Correspondence Address:
Prof. Surya Kant
Department of Respiratory Medicine, King George's Medical University, Lucknow, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijrc.ijrc_45_20

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Neurofibromatosis type 1 (NF1), also known as von Recklinghausen's disease, is an autosomal dominant genetic disorder. The incidence of NF is 1 in 3000 individuals. Approximately one-half of the cases are familial, while the remaining occur sporadically due to germ cell mutations. The clinical manifestation of NF1 may include a café-au-lait macule usually 5–15 mm in diameter, multiple neurofibromas, axillary or inguinal freckling, optic glioma, Lisch nodules, and a distinctive bony lesion. Although very rare, pulmonary manifestation in neurofibromatosis can occur in the form of mediastinal neurofibromas, pleural neurofibromas, tracheobronchial neurofibroma, interstitial lung disease, bullous lung disease, and cystic lung disease. Here, we present a rare case of pulmonary neurofibroma with bronchiectasis.

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