Results 101 to 110 of about 560,791 (351)

Characterizing the Healthcare Utilization and Costs of Hereditary Hemorrhagic Telangiectasia

American Journal of Hematology, EarlyView.
ABSTRACT Hereditary hemorrhagic telangiectasia (HHT) is the second‐most common inherited bleeding disorder worldwide, afflicting one in 4000–5000 people, and is the most morbid inherited bleeding disorder of women. HHT causes recurrent severe epistaxis, chronic gastrointestinal bleeding, heavy menstrual bleeding, and arteriovenous malformations in the ...
Hanny Al‐Samkari, Tracy J. Mayne, Misty Troutt, Hemant Patle, Marianne Clancy, Eric Duhaime   +5 more
wiley   +1 more source

Management of Pulmonary Arterial Hypertension

Journal of the American College of Cardiology, 2015
Pulmonary hypertension (PH) is common and may result from a number of disorders, including left heart disease, lung disease, and chronic thromboembolic disease. Pulmonary arterial hypertension (PAH) is an uncommon disease characterized by progressive remodeling of the distal pulmonary arteries, resulting in elevated pulmonary vascular resistance and ...
Vallerie V. McLaughlin, Sanjiv J. Shah, Marc Humbert, Rogério Souza   +3 more
openaire   +3 more sources

Sickle Cell Diastolic Cardiomyopathy and Mortality Risk: A Novel Echocardiographic Framework for Prognostic Stratification

American Journal of Hematology, EarlyView.
ABSTRACT Cardiovascular complications are the leading cause of mortality in sickle cell anemia (SCA) patients. While extensive data have identified diastolic dysfunction (DD) to increase morbidity and mortality, the unique hemodynamic conditions inherent to SCA challenge the current recommendations to assess diastolic function. Thus, there is an urgent
Théo Simon, Laurent Savale, Kristoffer Grundtvig Skaarup, Paul Breillat, Luu‐Ly Pham, Seyed‐Mehdi Nouraie, Niklas Dyrby Johansen, Jocelyn Inamo, Francois Lionnet, Gylna Loko, Christelle Chantalat, Anne Laure Pham Hung d’Alexandry d’Orengiani, Anoosha Habibi, Gonzalo de Luna, Sihem Iles, Frédéric Galactéros, Etienne Audureau, Tor Biering‐Sørensen, Pablo Bartolucci, Geneviève Derumeaux, Thomas d’Humières   +20 more
wiley   +1 more source

Osteopontin lung gene expression is a marker of disease severity in pulmonary arterial hypertension

Respirology (Carlton South. Print), 2019
Osteopontin (OPN) is a pleiotropic cytokine involved in the proliferation of pulmonary artery smooth muscle cells (PA‐SMC). OPN is upregulated in the lungs of patients with pulmonary hypertension (PH) associated with pulmonary fibrosis, suggesting that ...
M. Mura, M. Cecchini, Mariamma G. Joseph, J. Granton   +3 more
semanticscholar   +1 more source

Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. [PDF]

, 2013
BACKGROUND: Riociguat, a member of a new class of compounds (soluble guanylate cyclase stimulators), has been shown in previous clinical studies to be beneficial in the treatment of chronic thromboembolic pulmonary hypertension. METHODS: In this phase 3,
CHEST-1 Study Group,, D'Armini, AM, Fritsch, A, Ghofrani, HA, Grimminger, F, Hoeper, MM, Jansa, P, Kim, NH, Mayer, E, Neuser, D, Simonneau, G, Wang, C, Weimann, G, Wilkins, MR   +13 more
core   +1 more source

Characterization of Breakthrough Hemolysis in Patients With Paroxysmal Nocturnal Hemoglobinuria: An International Multicenter Experience

American Journal of Hematology, EarlyView.
ABSTRACT Breakthrough hemolysis (BTH) is defined as a hemolytic exacerbation in a patient with paroxysmal nocturnal hemoglobinuria (PNH) treated with complement inhibitors (CIs). In the current era of several terminal and proximal inhibitors, there are no guidelines for defining BTH and its severity, and clinical management is not standardized.
Bruno Fattizzo, Giacinto Luca Pedone, Elisabetta Metafuni, Eloise Beggiato, Valentina Giai, Esther Natalie Oliva, Antony Ricchiuti, Marta Bortolotti, Simona Raso, Daniela Carlino, Mara Memoli, Sofia Vecchi, Jennifer Vidler, Sukanya Gogoi, Shreya Goel, Anna Corby, Roochi Trikha, Sneha Balakrishnan, Francesco Passamonti, Anna Paola Iori, Simona Sica, Shreyans Gandhi, Wilma Barcellini, Austin Kulasekararaj   +23 more
wiley   +1 more source

A potential therapeutic role for angiotensin-converting enzyme 2 in human pulmonary arterial hypertension

European Respiratory Journal, 2018
Pulmonary arterial hypertension (PAH) is a deadly disease with no cure. Alternate conversion of angiotensin II (AngII) to angiotensin-(1–7) (Ang-(1–7)) by angiotensin-converting enzyme 2 (ACE2) resulting in Mas receptor (Mas1) activation improves rodent ...
A. Hemnes, A. Rathinasabapathy, E. Austin, E. Brittain, E. J. Carrier, Xinping Chen, J. Fessel, C. Fike, P. Fong, N. Fortune, Robert E. Gerszten, Jennifer A. Johnson, M. Kaplowitz, J. Newman, R. Piana, M. Pugh, T. Rice, I. Robbins, L. Wheeler, Chang Yu, J. Loyd, J. West   +21 more
semanticscholar   +1 more source

Stable isotope metabolomics of pulmonary artery smooth muscle and endothelial cells in pulmonary hypertension and with TGF-beta treatment. [PDF]

, 2020
Altered metabolism in pulmonary artery smooth muscle cells (PASMCs) and endothelial cells (PAECs) contributes to the pathology of pulmonary hypertension (PH), but changes in substrate uptake and how substrates are utilized have not been fully ...
D' Alessandro, Angelo, Freeman, Scott, Graham, Brian B, Gu, Sue, Hernandez-Saavedra, Daniel, Kassa, Biruk, Kumar, Rahul, Lee, Michael H, Mickael, Claudia, Reisz, Julie A, Sanders, Linda, Stenmark, Kurt R, Tuder, Rubin M   +12 more
core   +1 more source

Pulmonary Arterial Hypertension Onset in an Adult Woman With a TBX4 Likely Pathogenic Variant Following Imatinib Administration: A Case Report

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT The TBX4 gene has a critical importance in the development of the lower limbs and lungs. Pathogenic variants in this gene are associated with a variable spectrum of skeletal anomalies of the lower limb and pneumological manifestations, with dominant or recessive inheritance.
Simone Carbonera, Laura Scelsi, Alessandra Greco, Sandra Schirinzi, Annalisa Turco, Antonella Casella, Marta Carboni, Gaia Visani, Mauro Acquaro, Stefano Ghio, Silvia Kalantari, Enza Maria Valente, Fabio Sirchia   +12 more
wiley   +1 more source

Pulmonary hypertension in adolescents with sickle cell disease [PDF]

, 2016
Sickle cell disease consists of a group of disorders that have a similar mutation in at least one of the beta-globin chains of hemoglobin. This results in a change of the hemoglobin to sickle shaped cells when in the deoxygenated state.
Akinyemi, Katherine
core   +1 more source