Lung
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Epigenetics refers to changes in phenotype and gene expression that occur without alterations in DNA sequence. MicroRNAs are relatively recently discovered negative regulators of gene expression and act at the posttranscriptional level. ⋯ By better integrating network biology with evolving technologies in cell culture and in vivo experimentation, we will better understand epigenetic mechanisms of pulmonary hypertension and identify more diagnostic and therapeutic targets in pulmonary hypertension.
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Comparative Study
Relationship between quantitative CT metrics and pulmonary function in combined pulmonary fibrosis and emphysema.
Combined pulmonary fibrosis and emphysema (CPFE) is increasingly recognized, as current reports of its clinical features show. To determine CPFE's physiologic and radiologic features, we conducted quantitative assessment of computed tomography scans to compare with those of chronic obstructive pulmonary disease (COPD). ⋯ In CPFE, fibrosis and emphysema apparently existed independently, but both correlate with and likely contribute to the disproportionate reduction in gas exchange. Our study also suggested that pulmonary fibrotic changes may be more important contributors than emphysema for disease progression.
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Observational Study
Early initiation of prostacyclin in portopulmonary hypertension: 10 years of a transplant center's experience.
Portopulmonary hypertension (PoPH) is a subgroup of Group 1 pulmonary arterial hypertension (PAH) with particularly poor prognosis. Delay in initiation of parenteral therapy may be the reason for poor outcome. ⋯ Early initiation of parenteral prostacyclin therapy in PoPH patients at a single institution was associated with improved 5-year patient survival from diagnosis as compared to the REVEAL Registry of PoPH patients and allowed for clearance for transplant in 52 % of patients within 1 year.