Familial primary pulmonary hypertension

Known as: Familial Primary Pulmonary Hypertension [Disease/Finding], HPAH, Heritable Pulmonary Arterial Hypertension 
A rare, progressive, autosomal dominant inherited disorder. It is caused by mutation in the BMPR2 gene in most cases. It is characterized by… (More)
National Institutes of Health

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Highly Cited
2003
Highly Cited
2003
BACKGROUND Mutations of the transforming growth factor beta (TGFbeta) receptor components ENDOGLIN and ALK-1 cause the autosomal… (More)
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Highly Cited
2002
Highly Cited
2002
The recent discovery that sporadic and familial primary pulmonary hypertension can be associated with germline mutations of genes… (More)
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Highly Cited
2001
Highly Cited
2001
BACKGROUND Most patients with familial primary pulmonary hypertension have defects in the gene for bone morphogenetic protein… (More)
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Review
2001
Review
2001
The recent discoveries of the familial primary pulmonary hypertension gene and somatic mutations in key cell growth and cell… (More)
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Highly Cited
2000
Highly Cited
2000
Primary pulmonary hypertension (PPH), characterized by obstruction of pre-capillary pulmonary arteries, leads to sustained… (More)
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Highly Cited
2000
Highly Cited
2000
A clinically useful, treatment-based classification of pulmonary hypertension divides the disease into 5 distinct categories: (1… (More)
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Highly Cited
1997
Highly Cited
1997
Primary pulmonary hypertension (PPH)f an often fatal disease, is characterized by elevated pulmonary artery pressures in the… (More)
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Highly Cited
1997
Highly Cited
1997
BACKGROUND The pathogenesis of primary pulmonary hypertension (PPH) is unknown, although in some instances families with multiple… (More)
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Highly Cited
1995
Highly Cited
1995
The genetic basis of familial primary pulmonary hypertension (FPPH) is unknown, but the clinical and pathologic features are the… (More)
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1988
1988
We analyzed lung specimens from 23 affected members of 13 families with known familial primary pulmonary hypertension. In this… (More)
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