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Respir Med Case Rep · Jan 2016
Case ReportsA rare cause of cystic lung disease - Birt-Hogg-Dubé syndrome.
- P Minnis, P Riddell, and M P Keane.
- Dept. of Respiratory Medicine, St Vincent's University Hospital and School of Medicine and Medical Science, UCD Conway Institute, University College Dublin, Belfield, Dublin, 4, Ireland.
- Respir Med Case Rep. 2016 Jan 1; 18: 90-2.
AbstractBirt-Hogg-Dubé syndrome, initially described in 1977, is an autosomal dominant inherited condition characterised by basal pulmonary cysts often resulting in pneumothorax, renal tumours and cutaneous involvement. Lung cysts have been described in up to 90% of patients with a corresponding risk of pneumothorax of 50 times greater than the normal population. We describe here a case of Birt-Hogg-Dubé diagnosed in the 9th decade of life and discuss the radiological findings and clinical implications.
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