The purpose of this case study is to describe the physiological characteristics of a patient with the low-oxygen affinity Titusville hemoglobin variant. A 46-yr-old man with exertional dyspnea was diagnosed with a mediastinal lymphadenopathy of unknown origin and, to obtain definitive diagnosis by biopsy, underwent endobronchial ultrasound-guided transbronchial needle aspirate under sedation and video-assisted thoracoscopy under general anesthesia. High inspired fraction of oxygen ( ) was used to guarantee adequate oxygenation even during the one-lung ventilation needed for thoracoscopy. Following radial and pulmonary arterial catheterization, continuous mixed-venous oxygen saturation ( ), cardiac output, oxygen delivery (DO), oxygen consumption (V̇o), and oxygen extraction ratio (ERO) were measured. Serial blood gas analyses were obtained at different . Anesthesia and surgery were carried out safely. Data obtained during the clinical case were utilized to 1) construct an in vivo Titusville hemoglobin dissociation curve and 2) describe oxygen delivery and consumption of a human with Titusville mutation. Titusville hemoglobin showed relatively high P50 (i.e., 30 vs. normal of 27) and very low cooperativity (Hill coefficient of 1.45 vs. normal 2.27), which was compensated in our patient by increases in cardiac output, rather than by augmenting oxygen extraction.
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http://dx.doi.org/10.1152/japplphysiol.00860.2018 | DOI Listing |
Background: The introduction of proteasome inhibitors (PIs) and lenalidomide as treatment for newly diagnosed multiple myeloma (MM) has led to an increased population of lenalidomide-refractory patients. Limited data are available characterizing current treatments and outcomes in this difficult-to-treat population.
Methods: Individual patient-level data were analyzed from the treatment arms of multiple daratumumab studies, including APOLLO, CASTOR, CANDOR, EQUULEUS, ALCYONE, MAIA, GRIFFIN, POLLUX, and CASSIOPEIA.
Pulm Ther
November 2024
German Center for Lung Research (DZL), Giessen, Germany and University of Giessen and Marburg Lung Center (UGMLC), Giessen, Germany.
Introduction: Evidence for use of pulmonary arterial hypertension targeted-therapies in patients with chronic thromboembolic pulmonary hypertension (CTEPH) is limited. In MERIT-1, the endothelin receptor antagonist macitentan improved hemodynamic and functional parameters versus placebo in patients with inoperable CTEPH over a 24-week double-blind (DB) period. Its open-label (OL) extension study (MERIT-2) provides long-term safety/efficacy data.
View Article and Find Full Text PDFInt J Lab Hematol
June 2024
Department of Haematogenetics, Indian Council of Medical Research-National Institute of Immunohaematology (ICMR-NIIH), Mumbai, India.
The hemoglobin (Hb) variants are qualitative abnormalities due to production of structurally abnormal globin proteins. They are categorized based on the type of mutation present in the α1, α2, β, Gγ, Aγ and δ globin genes. So far, more than 1550 Hb variants are reported in the database.
View Article and Find Full Text PDFZhonghua Xue Ye Xue Za Zhi
December 2023
Department of Clinical Laboratory, the Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai 519000, China.
J Health Econ Outcomes Res
January 2024
Cytel Inc., Waltham, Massachusetts, USA.
Single-tablet combination therapies (STCTs) combine multiple drugs into one formulation, making drug administration more convenient for patients. STCTs were developed to address concerns with treatment adherence and persistence, but the impact of STCT use is not fully understood across indications. We conducted a systematic literature review (SLR) to examine STCT-associated outcomes across 4 evidence domains: clinical trials, real-world evidence (RWE), health-related quality of life (HRQoL) studies, and economic evaluations.
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