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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease idiopathic pulmonary arterial hypertension
Comorbidity C0009782|connective tissue disease
Sentences 8
PubMedID- 25842794 Material and method: in this retrospective descriptive study, the authors recruited patients with idiopathic pah (ipah) or pahassociated with connective tissue disease (pah-cnt) seen at the medicine department siriraj hospital between january 2005 and december 2011 for whom acute vasoreactivity test was indicated.
PubMedID- 25856504 Methods: plasma samples were collected from patients with idiopathic pulmonary arterial hypertension (ipah) and pah associated with connective tissue diseases (ctd-pah) attending two pulmonary hypertension referral centres (n = 95) and from age and gender matched healthy controls (n = 44).
PubMedID- 22872790 The pivotal subcutaneous treprostinil trial by simmoneau et al in 2002 enrolled 470 patients at 24 us centers and 16 international sites with pah, either primary pulmonary hypertension, that associated with connective tissue disease or congenital systemic-to-pulmonary shunts in a 12-week, double-blind, placebo-controlled trial.
PubMedID- 22043212 There are two randomized controlled trials with beraprost and a relatively large trial size of 130 and 116 patients, suffering from ipah and pah associated with connective tissue disease and chd, respectively.
PubMedID- 20170553 Articles were selected that described hemodynamic changes from baseline and considered trials among patients with ipah, or pah associated with connective tissue diseases (ctd), congenital heart disease (chd) or human immunodeficiency virus (hiv).
PubMedID- 21547012 Despite the heterogeneity of disease conditions that lead to ph, similar inflammatory cells types can be found in the plexiform vascular lesions in patients with ipah and ph associated with connective tissue disease and hiv.
PubMedID- 22034604 [3865] a prospective study in patients with ipah and pah associated with connective tissue disease showed that a tapse<1.8 cm was associated with lower invasively derived stroke volume index, higher right atrial pressure, greater rv dilatation, right to left heart disproportion, septal bowing and a higher incidence of pericardial effusion.
PubMedID- 24312667 In fact, recent data from patients with ph treated with vka demonstrated more major bleeding in ipah and pah associated with connective tissue disease than in cteph patients [7].

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