RT Journal Article
SR Electronic
T1 Quantitative CT scanning using a lung emphysema software package in familial Birt-Hogg-Dubé syndrome
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P751
VO 44
IS Suppl 58
A1 Sameh Samuel
A1 Deborah Yates
A1 Jonathan Lee
A1 Kathy Tucker
A1 Elizabeth Silverstone
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P751.abstract
AB Background:Birt-Hogg- Dubé syndrome (BHD) is a rare autosomal dominant disorder associated with skin lesions, cystic lung disease and renal tumours. Quantification of lung cysts volume can be performed using a lung emphysema software package, but correlation with lung function has not been previously examined.Aim:We aimed to correlate total lung emphysema volume with total lung capacity (TLC) in 6 patients with BHD.Method:Retrospective review of clinically gathered data, CT emphysema volume (using Philips Brilliance Emphysema Software), and lung function (using body plethysmography Sensormedics Vmax Encore Vision).Results:The use of Quantitative CT (QCT) emphysema package correlated with plethysmographic total lung capacity (TLC) [correlation coefficient 0.42] but FEV1/FVC did not seem to correlate with emphysema ratio [correlation coefficient -0.19].View this table:Patient's Data SummaryView this table:Conclusion:There is evidence of correlation between TLC and QCT lung volumes in Birt-Hogg-Dubé syndrome. Quantitative CT scanning requires further evaluation in this condition.Key WordsBirt-Hogg-Dubé Syndrome, Quantitative CT, Lung Function, Pneumothroax.