Idiopathic pulmonary arterial hypertension

Known as: Primary pulmonary hypertension, Idiopathic Pulmonary Hypertension, Hypertensions, Primary Pulmonary 
 
National Institutes of Health

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Autosomal dominant inheritanceDyspneaFamilial primary pulmonary hypertensionIdiopathic pulmonary hypertension
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Papers overview

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2012
2012
Leptin and regulatory T-lymphocytes in idiopathic pulmonary arterial hypertension.
Immune mechanisms and autoimmunity seem to play a significant role in idiopathic pulmonary arterial hypertension (IPAH… (More)
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Highly Cited
2010
Highly Cited
2010
Formation of plexiform lesions in experimental severe pulmonary arterial hypertension.
BACKGROUND The plexiform lesion is the hallmark of severe pulmonary arterial hypertension. However, its genesis and hemodynamic… (More)
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Review
2009
Review
2009
Updated clinical classification of pulmonary hypertension.
In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share… (More)
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Highly Cited
2009
Highly Cited
2009
Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era.
RATIONALE Pulmonary arterial hypertension in association with connective tissue disease (CTD-PAH) has historically had a poor… (More)
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Highly Cited
2006
Highly Cited
2006
Tricuspid annular displacement predicts survival in pulmonary hypertension.
RATIONALE Right ventricular (RV) function is an important determinant of prognosis in pulmonary hypertension. However… (More)
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Highly Cited
2006
Highly Cited
2006
Serotonin increases susceptibility to pulmonary hypertension in BMPR2-deficient mice.
Heterozygous germline mutations in the gene encoding the bone morphogenetic protein type II (BMPR-II) receptor underlie the… (More)
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Highly Cited
2005
Highly Cited
2005
Dysfunctional Smad signaling contributes to abnormal smooth muscle cell proliferation in familial pulmonary arterial hypertension.
Mutations in the bone morphogenetic protein type II receptor gene (BMPR2) are the major genetic cause of familial pulmonary… (More)
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Highly Cited
2003
Highly Cited
2003
Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement.
OBJECTIVE To determine whether there are factors, such as the diffusing capacity for carbon monoxide (DLCO) or pulmonary artery… (More)
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Review
2002
Review
2002
Cancer risk assessment, indicators, and guidelines for polycyclic aromatic hydrocarbons in the ambient air.
Polycyclic aromatic hydrocarbons (PAHs) are formed during incomplete combustion. Domestic wood burning and road traffic are the… (More)
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Highly Cited
2000
Highly Cited
2000
Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene.
Familial primary pulmonary hypertension is a rare autosomal dominant disorder that has reduced penetrance and that has been… (More)
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