Pulmonary features of Birt-Hogg-Dubé syndrome: cystic lesions and pulmonary histiocytoma.

  title={Pulmonary features of Birt-Hogg-Dub{\'e} syndrome: cystic lesions and pulmonary histiocytoma.},
  author={Sara Tomassetti and Angelo Carloni and Marco Chilosi and Adam Maffe and Silvana Ungari and Nicola Sverzellati and Gian Luca Casoni and Micaela Romagnoli and Ch Gurioli and Claudia Ravaglia and Venerino Poletti},
  journal={Respiratory medicine},
  volume={105 5},
BACKGROUND to describe clinical, radiologic and pathologic features of lung lesions in Birt-Hogg-Dubè syndrome (BHDS) (MIM 135150). METHOD review of 12 patients of BHDS from 3 unrelated Italian families evaluated at GB Morgagni Hospital, Forlì, from 2005 to 2010. RESULTS mean age (±SD) at diagnosis was 44.6 (±16) years, 8 (66%) were male. All three index cases presented with a history of recurrent pneumothorax and/or cystic lung lesions evaluated by CT scan request by referring pulmonary… CONTINUE READING


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