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PedAM

Pediatric Disease Annotations & Medicines




Disease pulmonary hypertension
Phenotype C0085786|idiopathic interstitial pneumonia
Sentences 4
PubMedID- 24937643 Rationale: pulmonary hypertension (ph) associated with fibrotic idiopathic interstitial pneumonia (iip; idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia) confers important additional morbidity and mortality.
PubMedID- 26583148 The more recent bphit (bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia) trial which evaluated the safety and clinical efficacy of bosentan in patients with ph and fibrotic idiopathic interstitial pneumonia revealed no demonstrable difference in symptoms, functional capacity, or pulmonary hemodynamics over a 16-week period [87].
PubMedID- 20113909 Results: the prevalence of pulmonary hypertension in patients with idiopathic interstitial pneumonia was 29% vs 64% in patients with connective tissue disease-interstitial pneumonia (p = 0.013).
PubMedID- 22171483 [relationship between combined emphysema, pulmonary hypertension and %fev1 in patients with idiopathic interstitial pneumonia].

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