Discrimination between COPD patients with and without alpha 1-antitrypsin deficiency using an electronic nose

Background and objective: To compare the volatile organic compound patterns of patients with COPD with and without alpha 1‐antitrypsin (AAT) deficiency using electronic nose technology. Methods: Exhaled breath condensate and pure exhaled breath of patients with COPD with (n = 10) and without (n = 23) AAT deficiency and healthy controls (n = 10) were analysed. The effect of human recombinant AAT on the volatile organic compound profile of 11 AAT‐deficient patients was also examined. Exhaled breath condensate and pure exhaled breath were measured using the Cyranose 320. Smell prints were analysed by linear discriminant analysis (LDA) using Mahalanobis distance (MD) and cross‐validation values (CVVs). Results: Smell prints of patients with AAT‐deficiency were different from those with COPD in exhaled breath condensate (LDA: P < 0.0001, sensitivity of 1.00, specificity of 1.00, CVV 82.0%, MD 2.37) and in pure exhaled breath (LDA: P < 0.0001, sensitivity of 1.00, specificity of 1.00, CVV 58.3%, MD 2.27). Smell prints of AAT‐deficient patients before and after human recombinant AAT augmentation were different (LDA: P = 0.001, sensitivity of 1.00, specificity of 1.00, CVV 53.3%, MD 1.79). Conclusions: An electronic nose can detect differences in smell prints of COPD patients with and without AAT deficiency. Augmentation therapy changes the volatile organic compound pattern. The electronic nose may be helpful in the diagnosis of AAT deficiency.     

Contribution of CT Quantified Emphysema,Air Trapping and Airway Wall Thickness on Pulmonary Function in Male Smokers With and Without COPD

Emphysema, airway wall thickening and air trapping are associated with chronic obstructive pulmonary disease (COPD). All three can be quantified by computed tomography (CT) of the chest. The goal of the current study is to determine the relative contribution of CT derived parameters on spirometry, lung volume and lung diffusion testing. Emphysema, airway wall thickening and air trapping were quantified automatically on CT in 1,138 male smokers with and without COPD. Emphysema was quantified by the percentage of voxels below –950 Hounsfield Units (HU), airway wall thickness by the square root of wall area for a theoretical airway with 10 mm lumen perimeter (Pi10) and air trapping by the ratio of mean lung density at expiration and inspiration (E/I-ratio). Spirometry, residual volume to total lung capacity (RV/TLC) and diffusion capacity (Kco) were obtained. Standardized regression coefficients (β) were used to analyze the relative contribution of CT changes to pulmonary function measures. The independent contribution of the three CT measures differed per lung function parameter. For the FEV1 airway wall thickness was the most contributing structural lung change (β = –0.46), while for the FEV₁/FVC this was emphysema (β = –0.55). For the residual volume (RV) air trapping was most contributing (β = –0.35). Lung diffusion capacity was most influenced by emphysema (β = –0.42). In a cohort of smokers with and without COPD the effect of different CT changes varies per lung function measure and therefore emphysema, airway wall thickness and air trapping need to be taken in account.     

Clinical Features of Polycythemia Vera and Essential Thrombocythemia in Japan: Retrospective Analysis of a Nationwide Survey by the Japanese Elderly Leukemia and Lymphoma Study Group

We conducted the first nationwide survey to clarify the clinical features, treatment methods, and prognoses for polycythemia vera (PV) and essential thrombocythemia (ET). A 1-page questionnaire was mailed to members of the Japanese Elderly Leukemia and Lymphoma Study Group (JELLSG). Surveys on 647 patients (PV, 266 patients; ET, 381 patients) were returned and analyzed. Thrombotic events at diagnosis and during follow-up occurred at rates of 15.4% and 8.5%, respectively, in PV cases and 17.6% and 8.7% in ET cases. Splenomegaly was observed in only 28.8% of PV patients and 10.8% of ET patients. The leukocyte alkaline phosphatase score was elevated in only 46.2% of PV patients. The incidences of abnormal karyotypes were less than 10% in both PV and ET cases. The rates of transformation to myelofibrosis were 2.6% in both PV and ET cases, and acute leukemia was noted in 1.1% of PV patients and 2.9% of ET patients. Prognostic factors were thrombotic history for PV and thrombotic history and age (>or=60 years) for ET. The present study clearly demonstrated clinical differences between Japanese and Western patients for PV and ET. Concerning the treatment of PV and ET, the study revealed considerable variation among Japanese hematologists. These results suggest the necessity of developing treatment guidelines according to risk stratification that are suitable for Japanese PV and ET patients.