Results 41 to 50 of about 1,381,406 (265)

Re‐Purposing Sapropterin (Kuvan) for ACTA2‐Related Multisystemic Smooth Muscle Dysfunction Syndrome: A Translational Mechanistic and First‐In‐Human Therapeutic Report

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Multisystemic smooth muscle dysfunction syndrome (MSMDS) is an ultra‐rare, ACTA2‐related disorder characterized by severe cerebrovascular disease, aortic aneurysms, and smooth muscle dysfunction. Using molecular dynamics simulations and in silico drug screening, we identified that sapropterin dihydrochloride (Kuvan) is a candidate capable of ...
Moran Hausman‐Kedem, Noa Bielopolski, Vijai Krishnan, Shane Wald‐Altman, Shelly I Shrian, Omer Bar‐Yosef, Livia Kapusta, Claire Shamber, Patricia L. Musolino, Miguel Weil   +9 more
wiley   +1 more source

Characteristics and Outcomes of Male Participants in a Multicenter Longitudinal Australian Study Cohort

Arthritis Care &Research, EarlyView.
Objective The aim of this study was to determine the differences in demographic, serologic, and clinical characteristics between male and female patients with systemic sclerosis (SSc) in an Australian cohort. Methods This was a retrospective observational study using data from the Australian Scleroderma Cohort Study.
Emily Lin, Dylan Hansen, Laura Ross, Gene‐Siew Ngian, Susanna Proudman, Gabor Major, Maryam Tabesh, Mandana Nikpour, Jennifer Walker, Lauren Host, Nava Ferdowsi, Alannah Quinlivan, Wendy Stevens, Joanne Sahhar, Diane Apostolopoulos   +14 more
wiley   +1 more source

Evaluation of code-based algorithms to identify pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension patients in large administrative databases

Pulmonary Circulation, 2020
Large administrative healthcare (including insurance claims) databases are used for various retrospective real-world evidence studies. However, in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, identifying patients ...
Viviane P. Sprecher, Eva-Maria Didden, Joel N. Swerdel, Audrey Muller   +3 more
doaj   +1 more source

Artificial Intelligence in Systemic Sclerosis: Clinical Applications, Challenges, and Future Directions

Arthritis Care &Research, EarlyView.
Systemic sclerosis (SSc) is a rare autoimmune disease defined by immune dysregulation, vasculopathy, and progressive fibrosis of the skin and internal organs. Despite advances in care, major complications such as interstitial lung disease (ILD) and myocardial involvement remain the leading causes of morbidity and mortality.
Cristiana Sieiro Santos, José Miguel Sequí‐Sabater, Diego Benavent   +2 more
wiley   +1 more source

Metabolomics of exercise pulmonary hypertension are intermediate between controls and patients with pulmonary arterial hypertension

Pulmonary Circulation, 2019
Mechanisms underlying pulmonary arterial hypertension (PAH) remain elusive. Pulmonary arterial hypertension and exercise PH share similar physiologic consequences; it is debated whether they share biologic mechanisms and if exercise PH represents an ...
Jason L. Sanders, Yuchi Han, Mariana F. Urbina, David M. Systrom, Aaron B. Waxman   +4 more
doaj   +1 more source

Adult congenital heart care in the COVID-19 era, and beyond: A call for actionKey Points

International Journal of Cardiology Congenital Heart Disease, 2020
While virus epidemics are nothing new to man, the scale, speed of global spread and immediacy of the COVID-19 pandemic have been truly unprecedented [1].
Michael A. Gatzoulis, Natali Chung, Paolo Ferrero, Massimo Chessa, George Giannakoulas, Aphrodite Tzifa, Gerhard P. Diller, Margarita Brida, Nada Al-Sakini   +8 more
doaj   +1 more source

Pulmonary arterial hypertension patients display normal kinetics of clot formation using thrombelastography

Pulmonary Circulation, 2021
Pulmonary arterial hypertension is characterized by endothelial dysfunction and microthrombi formation. The role of anticoagulation remains controversial, with studies demonstrating inconsistent effects on pulmonary arterial hypertension mortality ...
Mengyun Lu, Kevin P. Blaine, Ann Cullinane, Courtney Hall, Alina Dulau‐Florea, Junfeng Sun, Herman F. Chenwi, Grace M. Graninger, Bonnie Harper, Keshia Thompson, Janell Krack, Christopher F. Barnett, Samuel B. Brusca, Jason M. Elinoff, Michael A. Solomon   +14 more
doaj   +1 more source

Oxidative stress in patients with pulmonary hypertension

Advances in Redox Research, 2022
Rationale: Pulmonary arterial hypertension is a devastating cardiovascular disease that is progressive and fatal despite advances in therapy. Animal models have shown that oxidative stress may be pathogenic in pulmonary hypertension.
Tammy O. Wichman, Galo Martin Sanchez Palacios, Ross Davidson, Christopher S. Wichman, Matthew C. Zimmerman   +4 more
doaj   +1 more source

Immunosuppressive Drug Use in Limited Systemic Sclerosis: An International Survey

Arthritis Care &Research, EarlyView.
Objective Current guidelines recommend immunosuppressive treatment for diffuse cutaneous systemic sclerosis but are less clear on their use in limited cutaneous systemic sclerosis (lcSSc) in the absence of internal organ complications. We conducted an international survey to understand current immunosuppressive drug prescribing patterns in lcSSc ...
Sabrina Hoa, Yicheng Mu, Marie Hudson, Janet Pope   +3 more
wiley   +1 more source

Skeletal muscle dysfunctions in pulmonary arterial hypertension: Effects of aerobic exercise training

Frontiers in Physiology, 2023
Pulmonary arterial hypertension is associated with skeletal muscle myopathy and atrophy and impaired exercise tolerance. Aerobic exercise training has been recommended as a non-pharmacological therapy for deleterious effects imposed by pulmonary arterial
Filipe Rios Drummond, Luciano Bernardes Leite, Denise Coutinho de Miranda, Denise Coutinho de Miranda, Lucas Rios Drummond, Victor Neiva Lavorato, Leôncio Lopes Soares, Clóvis Andrade Neves, Antônio José Natali   +8 more
doaj   +1 more source