Lung function in Birt-Hogg-Dubé syndrome: a retrospective analysis of 96 patients.
Cécile DaccordVincent CottinG PrévotYurdagul UzunhanJean-François MornexP BonniaudRaphael BorieA BriaultM A Collonge-RameBruno CrestaniG DevouassouxO FreynetA GondouinP A HaussC KhouatraSylvie LeroySylvain Marchand-AdamCharles Hugo MarquetteI David MontaniJean-Marc NaccacheG NadeauN PoulalhonM Reynaud-GaubertM SalaunB WallaertJ F CordierM FaouziRomain Lazornull nullPublished in: Orphanet journal of rare diseases (2020)
Cystic lung disease in BHD does not affect respiratory function at baseline except for slightly increased RV and reduced DLco. No significant deterioration of lung function occurs in BHD over a follow-up period of 6 years.