Recognition of pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) (SSc-PAH) is very important , because SSc-PAH is highly prevalent and is a leading cause of mortality in patients with SSc. 1)2) Despite the use of novel therapeutic strategies, response to PAH-targeted therapy is still insufficient in SSc-PAH, which is associated with poor prognosis in SSc-PAH. 3) Ind...

Polycyclic aromatic hydrocarbons (PAH) were determined in two sediment cores in urban Lake Bled (NW Slovenia). In surface sediments of the deep western basin, the total PAH concentration was 1150 ng g dry weight (dw), whereas in shallower, nearshore Zaka Bay the PAH concentration was 1900 ng g dw. Fourto six-ring PAH were the most abundant compounds, suggesting that most PAH in Lake Bled sedime...

N-terminal pro-brain natriuretic peptide (NT-proBNP) is a marker of neurohormonal activation that is useful in the diagnosis and prognosis of various forms of pulmonary arterial hypertension (PAH). We sought to characterise and compare NT-proBNP in a cohort of PAH related to systemic sclerosis (PAH-SSc) and idiopathic PAH (IPAH) patients. NT-proBNP levels, collected from PAH-SSc and IPAH patien...

Background: We aimed to evaluate the expression of cysteine rich 61 (Cyr61) in patients with pulmonary arterial hypertension (PAH) as well as monocrotaline (MCT) induced PAH rat, and further investigate the effects and potential mechanisms of Cyr61 on the proliferation of pulmonary arterial smooth muscle cells (PASMCs). Methods and Results: Plasma samples were collected from 20 patients with id...

Pulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis (SSc). In clinical trials PAH-SSc has been grouped with other forms, including idiopathic PAH. The primary endpoint for most pivotal studies was improvement in exercise capacity. However, composite clinical endpoints that better reflect long-term outcome may be more meaningful. We discuss potential endpoints a...

Administrative claims studies do not adequately distinguish pulmonary arterial hypertension (PAH) from other forms of pulmonary hypertension (PH). Our aim is to develop and validate a set of algorithms using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes and electronic medical records (EMR), to identify patients with PAH. From January 2012 to Au...

The binding of labeled carcinogens [3H]benzo(a)pyrene [3H]dibenz(a,/i)anthracene, and [3H]dibenz(a,c)anthracene to nuclear chromatin was examined in AKR mouse embryo cells grown in culture. The influence of unlabeled weakly and strongly carcinogenic polycyclic aromatic hydrocar bons (PAH) and the metabolic inhibitor 7,8-benzoflavone on the binding of labeled PAH to nuclear chromatin was examine...

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by vascular remodeling of pulmonary arteries (PAs) and increased vascular resistance in the lung. Monocrotaline (MCT), a toxic alkaloid, is widely used for developing rat models of PAH caused by injury to pulmonary endothelial cells; however, characteristics of vascular functions in MCT-induced PAH vary and are not ful...

Tetrahydrobiopterin (BH4)-responsive phenylalanine hydroxylase (PAH) deficiency is a recently recognized variant of phenylketonuria, with a probable multifactorial molecular basis. In this study we have investigated the effect of BH4 on PAH gene expression in human hepatoma. Our results show that increased BH4 levels result in an enhancement of PAH activity and PAH protein, due to longer turnov...