Prevalence of pulmonary hypertension in end-stage cystic fibrosis and correlation with survival.

Background: Limited information is available about the prevalence of pulmonary hypertension diagnosed by right heart catheterization (RHC) in patients with cystic fibrosis being evaluated for lung transplantation. It is unclear whether there are factors that can predict the presence of pulmonary hypertension and whether the presence of pulmonary hypertension influences patient outcomes.

Methods: The study included 57 unique and consecutive adult patients (33 women) with cystic fibrosis who underwent lung transplant evaluation at the University of Florida.

Discrepancies between clinical and autopsy diagnoses in lung transplant recipients.

We sought to investigate the role of autopsy diagnoses in lung transplantation by comparing the clinically derived cause of death with autopsy deduced cause of death in a cohort of lung transplant recipients. We retrospectively reviewed all consecutive autopsy findings on lung transplant recipients transplanted between March 1994 and March 2007. We reviewed medical records and our lung transplant database to determine the clinical diagnosis of cause of death based on the clinical assessment and discharge summary at the time of death.

Pleural mesothelial cell transformation into myofibroblasts and haptotactic migration in response to TGF-beta1 in vitro.

Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology characterized by the development of subpleural foci of myofibroblasts that contribute to the exuberant fibrosis noted in the pulmonary parenchyma. Pleural mesothelial cells (PMC) are metabolically dynamic cells that cover the lung and chest wall as a monolayer and are in intimate proximity to the underlying lung parenchyma. The precise role of PMC in the pathogenesis of pulmonary parenchymal fibrosis remains to be identified.

Left pneumonectomy in a patient with a chronically infected allograft.

We describe a young man with cystic fibrosis who underwent bilateral sequential lung transplantation (BSLT) and a subsequent right single-lung re-transplant for bronchiolitis obliterans syndrome (BOS). Destruction of the retained left lung with recurrent pneumonia, worsening bronchiectasis and abscess formation was treated with a left lower lobectomy and a subsequent complete pneumonectomy. The patient tolerated the procedures and is alive and well 18 months after left pneumonectomy.