PubMed · 15408999
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T BADGER, R H SWEET. 1950-01-26. Pulmonary emphysema diffuse, severe.. https://doi.org/10.1056/nejm195001262420407
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Mounier-Kuhn syndrome is a rare condition characterized by marked dilation of the trachea and main bronchi resulting in bronchiectasis and emphysema. We report a case in which a patient underwent successful double lung transplantation for COPD that was found on pathologic examination of the explanted lungs to be Mounier Kuhn syndrome. To our knowledge this is the first case reporting lung transplantation in this syndrome.
Long-range diffusivity of hyperpolarized 3He gas was measured from the decay rate of sinusoidally modulated longitudinal nuclear magnetization in three normal donor and nine severely emphysematous explanted human lungs. This (long-range) diffusivity, which we call Dsec, is measured over seconds and centimeters and is approximately 10 times smaller in healthy lungs (0.022 cm2/s) than the more traditionally measured Dmsec, which is measured over milliseconds and submillimeters. The increased restriction of Dsec reflects the complex, tortuous paths required to navigate long distances through the maze of branching peripheral airways. In emphysematous lungs, Dsec is substantially increased, with some regions showing nearly the unrestricted value of the self-diffusion coefficient (0.88 cm2/s for dilute 3He in air, a 40-fold increase). This suggests the presence of large collateral pathways opened by alveolar destruction that bypass the airways proper. This destruction was confirmed by comparison with histology in seven lungs and by removal of trapped gas via holes in the pleural surface in five lungs.
BACKGROUND: The purpose of this study was to assess the quality of sleep and extent of respiratory disorders in patients awaiting lung transplantation as compared with a control group. METHODS: From September 2003 to November 2003, 17 clinically stable patients on the waiting list for lung transplantation and 14 healthy controls (with similar age, gender, and body mass index) were studied. Diagnostic polysomnography (PSG) was carried out for all subjects. RESULTS: Seventeen patients were included, 15 men and 2 women, aged 51 +/- 14 years. The indication for lung transplantation was emphysema in 7 cases, pulmonary fibrosis in 6, and "other" in 4. Patients awaiting lung transplantation had the following respiratory values: mean FEV1, 1105 mL (34% of predicted); PaO2, 54 mm Hg; and PaCO2, 44 mm Hg. Significant differences were found among the waiting-list patients in terms of predominance of light sleep, wakeful periods, and phase changes per sleep-hour, as compared with the control group. The recording of the respiratory events showed an apnea-hypopnea index of 6.13, sleeping time with SaO2 <90% of 1.80%, and a mean number of significant desaturations (<4%) of 6.38. There were no statistically significant differences with respect to the control group. CONCLUSIONS: Poor quality of sleep was observed in patients awaiting lung transplantation as compared with a healthy control group. There was no evidence of more respiratory events or significant desaturations in these patients, probably due to the provision of supplementary oxygen therapy during the PSG.