نتایج جستجو برای: idiopathic pulmonary arterial hypertension ipah
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Pulmonary arterial hypertension (PAH) is a multifactorial life-threatening disease, characterized by high pulmonary artery pressure, with an ultimate right heart failure. Without treatment, death occurs within 3 years of diagnosis. PAH is characterized by abnormal remodelling of small, peripheral resistance vessels in the lung involving proliferation and migration of vascular smooth muscle, end...
Zhang S, Patel HH, Murray F, Remillard CV, Schach C, Thistlethwaite PA, Insel PA, Yuan JX. Pulmonary artery smooth muscle cells from normal subjects and IPAH patients show divergent cAMP-mediated effects on TRPC expression and capacitative Ca entry. Am J Physiol Lung Cell Mol Physiol 292: L1202–L1210, 2007. First published December 22, 2006; doi:10.1152/ajplung.00214.2006.— Pulmonary vascular r...
Nonphagocytic NADPH oxidases have recently been suggested to play a major role in the regulation of physiological and pathophysiological processes, in particular, hypertrophy, remodeling, and angiogenesis in the systemic circulation. Moreover, NADPH oxidases have been suggested to serve as oxygen sensors in the lung. Chronic hypoxia induces vascular remodeling with medial hypertrophy leading to...
A subgroup of patients with idiopathic pulmonary arterial hypertension (IPAH) has severely reduced diffusing capacity of the lung for carbon monoxide (DLCO) and poor prognosis. Their characteristics are currently unknown. The aim of this study is to contrast clinical characteristics and treatment responses of IPAH-patients with a severely reduced and more preserved DLCO. Retrospectively, 166 IP...
BACKGROUND Gene expression profiling of peripheral blood mononuclear cells (PBMCs) is a powerful tool for the identification of surrogate markers involved in disease processes. The hypothesis tested in this study was that chronic exposure of PBMCs to a hypertensive environment in remodeled pulmonary vessels would be reflected by specific transcriptional changes in these cells. METHODOLOGY/PRI...
Idiopathic pulmonary arterial hypertension (IPAH) is a fatal disease with a poor prognosis and the molecular pathways underlying the pathogenesis of IPAH are not fully understood. In the present study, the long non-coding RNA (lncRNA) and mRNA expression profiles of lymphocytes obtained from 12 IPAH patients and 12 healthy controls were analyzed using Arraystar Human lncRNA Microarray v2.0, and...
BACKGROUND Idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) share important pathogenic and clinical features. BMPR2 mutations are important in the pathogenesis of IPAH, but little is known about the genetic background in CTEPH. OBJECTIVE To search for mutations and polymorphisms in genes involved in the BMPR2, serotonin and nitric oxi...
OBJECTIVE To review the current pharmacotherapy for idiopathic pulmonary arterial hypertension (IPAH). METHODS A search of the primary literature was conducted by using MEDLINE, the National Institutes of Health medical research Web site (www.clinicaltrials.gov), and the United States Food and Drug Administration's Center for Drug Evaluation and Research Web site (www.fda.gov/cder). RESULTS...
RATIONALE Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary arterial hypertension (PAH). Oral anticoagulation is confined to patients with idiopathic PAH (IPAH), but no oral anticoagulation has been recommended for PVOD, because occult pulmonary hemorrhage was a common finding in PVOD. PATIENT CONCERNS We report a case of PVOD, who was misdiagnosed as IPAH for 5 years with w...
RATIONALE Pulmonary arterial hypertension (PAH) may be classified as idiopathic (IPAH) or familial (FPAH) or associated with various conditions and exposures such as dexfenfluramine intake (Dex-PAH) or systemic sclerosis (SSc-PAH). Because fibroblast dysfunction has been identified in SSc and IPAH and antifibroblast antibodies (AFAs) with a pathogenic role have been detected in the serum of SSc...
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