Results 141 to 150 of about 423,899 (348)

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

Transmembrane protein 16A/anoctamin 1 inhibitor T16A-A01 reversed monocrotaline-induced rat pulmonary arterial hypertension

Pulmonary Circulation, 2020
Transmembrane protein 16A was involved in the development of the monocrotaline-induced pulmonary arterial hypertension model through ERK1/2 activation, and it was considered as potential target for pulmonary arterial hypertension treatment.
Jianye Xie, Wenyuan Liu, Wenjing Lv, Xiaohua Han, Qingnuan Kong, Yuhui Wu, Xin Liu, Ying Han, Chunying Shi, Xiujuan Jia   +9 more
doaj   +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

Plasma adrenomedullin peptides and precursor levels in pulmonary arterial hypertension disease severity and risk stratification

Pulmonary Circulation, 2020
Adrenomedullin is a potent vasodilatory peptide, linked to pulmonary arterial hypertension pathology. Proximity extension assays were utilized to study plasma biomarkers related to vasoregulation, with focus on adrenomedullin peptides and precursor ...
Habib Bouzina, Göran Rådegran
doaj   +1 more source

Role of extracellular matrix in the pathogenesis of pulmonary arterial hypertension.

American Journal of Physiology. Heart and Circulatory Physiology, 2018
Pulmonary arterial hypertension (PAH) is characterized by remodeling of the extracellular matrix (ECM) of the pulmonary arteries with increased collagen deposition, cross-linkage of collagen, and breakdown of elastic laminae.
T. Thenappan, Stephen Y. Chan, E. Weir
semanticscholar   +1 more source

Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor [PDF]

, 2002
BACKGROUND: Mutations in the type II receptor for bone morphogenetic protein (BMPR-II), a receptor member of the transforming growth factor-beta (TGF-beta) superfamily, underlie many familial and sporadic cases of primary pulmonary hypertension (PPH ...
Atkinson, Carl, Machado, Rajiv D., Morrell, Nicholas W., Stewart, Susan, Thomson, Jennifer R., Trembath, Richard C., Upton, Paul D.   +6 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

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  

Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

European Respiratory Journal, 2019
Background Current pulmonary hypertension treatment guidelines recommend use of a risk stratification model encompassing a range of parameters, allowing patients to be categorised as low, intermediate or high risk. Three abbreviated versions of this risk
M. Humbert, H. Farber, H. Ghofrani, R. Benza, D. Busse, C. Meier, M. Hoeper   +6 more
semanticscholar   +1 more source

Genotype–Phenotype Correlation in TTC7A‐Associated Gastrointestinal Defects and Immunodeficiency Syndrome 1

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Gastrointestinal defects and immunodeficiency syndrome 1 (GIDID1) is a rare autosomal recessive disorder caused by biallelic variants in TTC7A. GIDID1 is characterized by a broad clinical spectrum ranging from very early‐onset inflammatory bowel disease (VEOIBD) to multiple intestinal atresia (MIA) with or without immunological manifestations.
Julia Imhoff, Hans Christian Schmidt, Matthias Hans Belau, Johanna Hagens, Mario Lange, Daniel Tegtmeyer, Konrad Reinshagen, Frederike Leonie Harms, Christian Tomuschat   +8 more
wiley   +1 more source