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Benefits and Limitations of Long-Term Eculizumab Treatment for Paroxysmal Nocturnal Hemoglobinuria (PNH): Real-World Data from Large Cohort Study in Russia
Conclusions: The results of the study show both the high efficacy and limitations of treatment with eculizumab for PNH in real-world practice. Prospectively confirmed significant improvement of the overall survival on eculizumab stress the need for faster and wider access to costly therapy. Nevertherless, a number of limitations, including BTH and extravascular hemolysis, lack of control of bone marrow failure and further clonal evolution, determine the relevance of next-generation complement inhibitors and risk-adjusted allogeneic HSCT as a curative option.DisclosuresKulagin: Alexion Pharmaceuticals, Inc: Consultancy, Honoraria.
Source: Blood - November 21, 2018 Category: Hematology Authors: Kulagin, A., Klimova, O., Rudakova, T., Golubovskaya, I., Ivanova, M., Smirnova, A., Lapina, A., Bykova, T., Babenko, E., Popova, M., Dobronravov, V., Afanasyev, B. Tags: 508. Bone Marrow Failure: Poster II Source Type: research

Echocardiographic assessment in lowlanders vs. highlanders living above 2500m
Conclusion: Chronic exposure to hypoxia in HL promotes a rise in pulmonary artery pressure and a decreased right heart function compared to LL.
Source: European Respiratory Journal - November 19, 2018 Category: Respiratory Medicine Authors: Lichtblau, M., Saxer, S., Bader, P., Furian, M., Scheiwiller, P., Mademilov, M., Estebesova, B., Osmonov, B., Marazhapov, N., Sheraliev, U., Sooronbaev, T., Bloch, K., Ulrich, S. Tags: Pulmonary hypertension Source Type: research

Right heart function and pulmonary pressure in asthma patients during 17 days at high-altitude
Conclusions: In otherwise healthy asthmatics exposure to high altitude induces a significant increase in mPAP and heart rate with a decrease in SI resulting in an unchanged CI. During the 17 days at 3200m there is no further change in the indices of RVF despite increasing SpO2. Thus, respiratory acclimatization is more rapid than the one of the pulmonary circulation.
Source: European Respiratory Journal - December 6, 2017 Category: Respiratory Medicine Authors: Bader, P. R., Lichtblau, M., Saxer, S., Schneider, S., Appenzeller, P., Furian, M., Estebesova, B., Sooronbaev, T., Bloch, K. E., Ulrich, S. Tags: Pulmonary Circulation and Pulmonary Vascular Disease Source Type: research