Results 181 to 190 of about 21,756 (223)

Heterogeneity of Pathologic Lesions in Familial Primary Pulmonary Hypertension

The American Review of Respiratory Disease, 1988
Abstract We analyzed lung specimens from 23 affected members of 13 families with known familial primary pulmonary hypertension. In this heritable disease, the pathogenetic basis of disease should be distinct within families, and the pathologic lesions should reflect the underlying biologic mechanisms.
James Loyd, John Newman, Giuseppe G Pietra   +2 more
exaly   +3 more sources

BMPR2 mutations found in Japanese patients with familial and sporadic primary pulmonary hypertension

Human Mutation, 2004
Primary pulmonary hypertension (PPH) is a potentially lethal disorder, in which heterozygous mutations within the bone morphogenetic protein type II receptor (BMPR2) gene (BMPR2) have been identified. We conducted a molecular study of BMPR2 mutations in 4 Japanese families with familial PPH and 30 Japanese patients with sporadic PPH, and found 13 ...
Hiroko, Morisaki, Norifumi, Nakanishi, Shingo, Kyotani, Atsushi, Takashima, Hitonobu, Tomoike, Takayuki, Morisaki   +5 more
exaly   +3 more sources

The Familial Occurrence of Primary Pulmonary Hypertension

Annals of Internal Medicine, 1966
Excerpt Primary pulmonary hypertension is a disease characterized by increased arterial pressure in the pulmonary circulation caused by changes which appear to be intrinsic to the pulmonary vascula...
J D, Rogge, M E, Mishkin, P D, Genovese
openaire   +2 more sources

Familial Primary Pulmonary Hypertension

Chest, 1994
Often, insights into a disease are gained by studying the exceptional cases, the outliers. For the population with primary pulmonary hypertension (PPH), it is the patients with the familial or hereditary form that are the outliers, representing only 6 percent of the recent NHLBI PPH Registry population.
openaire   +2 more sources

FAMILIAL PRIMARY PULMONARY HYPERTENSION AND ASSOCIATED OCULAR FINDINGS

Retina, 2001
Familial primary pulmonary hypertension (PPH) is a rare, fatal, autosomal dominant disease that results in right heart failure from idiopathic obliteration of the pulmonary arteries. Patients develop stagnation of venous blood flow and elevated venous pressure.The authors retrospectively reviewed the clinical records of three patients diagnosed with ...
Saran BR, Brucker AJ, BANDELLO , FRANCESCO, Verougstraete C.   +3 more
openaire   +4 more sources

Familial Primary Pulmonary Hypertension

Southern Medical Journal, 1974
Primary pulmonary hypertension occurring in a 21-year-old woman, her father, and her father's sister is documented. The diagnosis was established by cardiac catheterization in all of the patients. Pulmonary arteriography was done in two cases and a postmortem examination in one.
openaire   +2 more sources

Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31–32

Nature Genetics, 1997
Primary pulmonary hypertension (PPH), an often fatal disease, is characterized by elevated pulmonary artery pressures in the absence of a secondary cause. Endovascular occlusion in the smallest pulmonary arteries occurs by proliferation of cells and matrix, with thrombus and vasospasm.
W C, Nichols, D L, Koller, B, Slovis, T, Foroud, V H, Terry, N D, Arnold, D R, Siemieniak, L, Wheeler, J A, Phillips, J H, Newman, P M, Conneally, D, Ginsburg, J E, Loyd   +12 more
openaire   +2 more sources

[Primary familial pulmonary arterial hypertension].

Revue des maladies respiratoires, 1998
The authors present the case of three young female patients, of the same family, with all quickly fatal Primary Pulmonary Hypertension (PPH). Their clinical signs and paraclinical investigations were suggestive of a PPH and excluded all others causes of Pulmonary Hypertension.
A, Scherpereel, F, Steenhouwer, P, Quandalle, S, Dennetière, Y, Haftel, N, Declercq, J M, Demarcq   +6 more
openaire   +1 more source

Mapping of Familial Primary Pulmonary Hypertension Locus (PPH1) to Chromosome 2q31-q32

Circulation, 1997
Background The pathogenesis of primary pulmonary hypertension (PPH) is unknown, although in some instances families with multiple affected members suggest a genetic etiology. Methods and Results We used microsatellite markers and linkage analysis in a large family with PPH to ...
J H, Morse, A C, Jones, R J, Barst, S E, Hodge, K C, Wilhelmsen, T G, Nygaard   +5 more
openaire   +2 more sources

Familial primary pulmonary hypertension: clinical patterns.

The American review of respiratory disease, 1984
We recently identified a family with 6 deaths in 2 generations from primary pulmonary hypertension. The disease was undiagnosed in several of them, and the family members, geographically separated, were unaware that there was a familial disease. We hypothesized that familial primary pulmonary hypertension (FPPH) might be labeled as nonfamilial in ...
J E, Loyd, R K, Primm, J H, Newman
openaire   +1 more source