Rare diseases bullet 5: Primary pulmonary hypertension

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Evaluation of Pulmonary Hypertension In First Degree Relatives Of Patients With Primary Pulmonary Hypertension

Background: Pulmonary hypertension (PH) was defined for the first time in 1951 as primary pulmonary hypertension (PPH). Some studies emphasized on the role of genetics in the development of pulmonary hypertension in family members of affected patients. So, in this study we evaluated the prevalence of pulmonary hypertension in first degree family of patients with documented PPH. Methods: In thi...

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[Primary pulmonary hypertension in Sjögren's syndrome: a rare association].

Primary Sjögren’s syndrome (SS) is rarely associated with pulmonary hypertension (PH). In connective tissue diseases, the development of PH is usually due to interstitial lung involvement. We present the case of a 40-year-old female who came to the Rheumatology department in 2002 with xerophthalmia of 5 years’ evolution to which xerostomia and arthralgia had been added in the last 6 months, wit...

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Is resveratrol the magic bullet for pulmonary hypertension?

Pulmonary hypertension (PHTN) remains a disease with high morbidity and mortality and no known cure. The World Health Organization has categorized PHTN into 5 clinical classifications, as follows: (1) pulmonary artery hypertension; (2) PHTN with left heart disease; (3) PHTN with respiratory disease; (4) PHTN caused by chronic thrombotic/embolic disease; and (5) miscellaneous PHTN.1 Despite the ...

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Primary pulmonary hypertension.

Primary pulmonary vascular sclerosis was first fully described by Brenner (1935) who stressed the presence of hypertrophy of the right, but not of the left ventricle; he concluded his description with the opinion that primary sclerosis is not a pathological entity but includes several conditions. Other authors, notably De Navasquez et al. (1940), preferred the term " primary or idiopathic right...

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[Primary pulmonary hypertension].

Clinical, pharmacologic, and pathologic findings presented are those in a 12-year-old boy with primary pulmonary hypertension associated with medial hypertrophy of the pulmonary muscular arteries and arterioles. Intrapulmonary arterial administration of acetylcholine, tolazoline (Priscoline), or isoproterenol resulted in a fall in pulmonary vascular resistance and pressure. Perforation of the r...

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ژورنال

عنوان ژورنال: Thorax

سال: 1999

ISSN: 0040-6376

DOI: 10.1136/thx.54.12.1107