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...

Vascular remodeling in idiopathic pulmonary arterial hypertension (IPAH) involves hyperproliferative and apoptosis-resistant pulmonary artery endothelial cells. In this study, we evaluated the relative contribution of bone marrow-derived proangiogenic precursors and tissue-resident endothelial progenitors to vascular remodeling in IPAH. Levels of circulating CD34+ CD133+ bone marrow-derived pro...

Pulmonary arterial hypertension (PAH) is a rare disorder that may be hereditable (HPAH), idiopathic (IPAH), or associated with either drug-toxin exposures or other medical conditions. Familial cases have long been recognized and are usually due to mutations in the bone morphogenetic protein receptor type 2 gene (BMPR2), or, much less commonly, 2 other members of the transforming growth factor-b...

Survival rates for patients with idiopathic pulmonary arterial hypertension (IPAH) have improved with the introduction of PAH-specific therapies. However, the time between patient-reported onset of symptoms and a definitive diagnosis of IPAH is consistently delayed. We conducted a retrospective, multi-center, descriptive investigation in order to (a) understand what factors contribute to persis...

Patient Cause of PH Age (years) Gender mPAP (mm Hg) RAP (mm Hg) CO (L.min) TPR (dyne.sec.cm) 1 IPAH 27 F 55 13 2,9 1733 2 IPAH 56 M (64) (15) (5.6) (923) 3 IPAH 42 M 30 NA 9,2 264 4 EAA 44 M 33 22 6,2 422 5 COPD 59 M 30 15 7,2 330 6 sarcoidosis 44 M 48 11 NA NA 7 IPAH 41 F (60) (13) (3.2) (1481) 8 IPAH 37 F 45 14 5,4 662 9 IPF 64 M 51 8 5,1 805 10 EAA 52 M 43 11 4,5 764 11 PAH-CHD 45 V (71) (14...

I diopathic pulmonary arterial hypertension (IPAH) used to be called primary pulmonary hypertension. Both terms indicate that we do not yet fully understand the aetiology of the disease. A clue to the underlying mechanisms lies in the observation that the appetite-suppressant drugs aminorex [1, 2] and fenfluramine [3] were both associated with a marked increase in the incidence of pulmonary art...

BACKGROUND Acute pulmonary vasoreactivity testing has been recommended in the diagnostic work-up of patients with idiopathic pulmonary arterial hypertension (IPAH). Pulmonary arteriolar capacitance (Cp) approximated by stroke volume divided by pulmonary pulse pressure (SV/PP) is considered as an independent predictor of mortality in patients with IPAH. OBJECTIVES We sought to evaluate any dif...

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...

Bacillary dysentery caused by Shigella species is a public health problem in developing countries including Bangladesh. Although, shigellae-contaminated food and drinks are often the source of the epidemic's spread, the possible presence of the pathogen and transmission of it through environmental waters have not been adequately examined. We analyzed surface waters collected in Dhaka, Banglades...

Idiopathic pulmonary arterial hypertension (IPAH) is a rare and progressive disease of unknown pathogenesis. Vascular remodeling due to excessive proliferation of pulmonary arterial smooth muscle cells (PASMCs) is a critical pathogenic event that leads to early morbidity and mortality. The excessive cell proliferation is closely linked to the augmented Ca2+ signaling in PASMCs. More recently, w...