Cystic lung disease in Birt-Hogg-Dube syndrome.

Background: To describe the clinical, radiologic, and histopathologic aspects of cystic lung disease occurring in patients with Birt-Hogg-Dubé (BHD) syndrome, a rare, inheritable, multisystem disorder.

Methods: We retrospectively reviewed five patients with BHD syndrome evaluated at the Mayo Clinic Rochester from 1998 through 2005.

Results: Mean age (+/- SD) at the time of pulmonary evaluation was 56.

Assessing need for long-term oxygen therapy: a comparison of conventional evaluation and measures of ambulatory oximetry monitoring.

Background: Appropriate identification of hypoxic patients with chronic obstructive pulmonary disease (COPD) is important because of the demonstrated survival benefit of long-term oxygen therapy (LTOT) and its associated cost. Resting oxygen saturation (measured via pulse oximetry [S(pO2)]) and lowest exercise S(pO2) (during a 6-min walk test) is the standard method of determining LTOT requirements, but that method does not measure the patient's oxygenation during sleep or activities of daily living. We hypothesized that values obtained via the standard method would correlate poorly with values obtained via ambulatory oximetry monitoring.