Effect of 6-min Walk Test on pro-BNP Levels in Patients with Pulmonary Arterial Hypertension.

Background: Plasma pro-BNP (brain natriuretic peptide) levels are often elevated in response to right ventricular (RV) volume and pressure overload, parameters potentially affected by exercise. Plasma pro-BNP levels change in association with long-term changes in pulmonary hemodynamics, thereby serving as a potential biomarker in pulmonary arterial hypertension (PAH). The 6-min Walk Test (6MWT) and pro-BNP level are often checked in a single office visit.

Long-term outcomes of the bronchial artery embolization are diagnosis dependent.

Background: Bronchial artery embolization (BAE) is an established, safe, and effective procedure for the treatment of hemoptysis but long-term outcomes of the BAE have never been investigated before.

Objectives: To retrospectively analyze long-term outcomes of the BAE.

Materials And Methods: A retrospective chart analysis was done from the hospital central database for all patients undergoing the BAE over a consecutive 14-year period (January 2000-February 2014).

Pan-Resistant Achromobacter xylosoxidans and Stenotrophomonas maltophilia Infection in Cystic Fibrosis Does Not Reduce Survival After Lung Transplantation.

Background: The number of cystic fibrosis (CF) patients undergoing lung transplantation continues to grow, as does the prevalence of multidrug-resistant (MDR) gram-negative rods. However, the posttransplant survival of patients with MDR pathogens, specifically pan-resistant Achromobacter xylosoxidans and Stenotrophomonas maltophilia, is poorly characterized.

Methods: This was a retrospective review of CF patients (n = 186; all age, > 16 years) transplanted at the University of North Carolina from 1990 through 2013.

Safety and feasibility of obtaining wedged pulmonary artery samples and differential distribution of biomarkers in pulmonary hypertension.

This study aims to evaluate the safety and feasibility of obtaining wedged pulmonary artery (PA) samples and investigate the differential vascular beds' distribution of select inflammatory and cellular adhesion molecules that are implicated in pulmonary arterial hypertension (PAH) pathogenesis. This is a cross-sectional study of adult patients. Serum samples were simultaneously drawn from three different vascular sites during right heart catheterization as part of PAH evaluation: The superior vena cava, distal pulmonary artery prior to wedging, and distal pulmonary artery after (and distal to) wedging.

Donor-specific antibodies are associated with antibody-mediated rejection, acute cellular rejection, bronchiolitis obliterans syndrome, and cystic fibrosis after lung transplantation.

Background: Lung transplantation is limited by chronic lung allograft dysfunction. Acute cellular rejection (ACR) is a risk factor for allograft dysfunction; however, the role of antibody-mediated rejection (AMR) is not well characterized.

Methods: This was a retrospective review from 2007 to 2011 of lung transplant recipients with human leukocyte antigen (HLA) antibody testing using Luminex (Luminex Corp, Austin, TX) single-antigen beads.

Thermodilution and Fick cardiac outputs differ: impact on pulmonary hypertension evaluation.

Background: The relationship between thermodilution and indirect Fick cardiac output determination methods has not been well described.

Objective: To describe the relationship between these two cardiac output determination methods in patients evaluated for pulmonary hypertension and to highlight potential clinical implications.

Methods: A retrospective review of charts of all adult patients who underwent a right heart catheterization (RHC) between January 1, 2007 and November 10, 2010, and participated in the pulmonary hypertension program of the pulmonary division at an academic institution was conducted.

Increased plasma mannose binding lectin levels are associated with bronchiolitis obliterans after lung transplantation.

Background: Long-term lung allograft survival is limited by bronchiolitis obliterans syndrome (BOS). Mannose binding lectin (MBL) belongs to the innate immune system, participates in complement activation, and may predispose to graft rejection. We investigated mannose binding (MBL) during cold ischemia and in tissue samples from explanted lungs with BOS, and assessed MBL and complement proteins in plasma post-lung transplantation relative to BOS staging.

European cystic fibrosis bone mineralisation guidelines.

Patients with cystic fibrosis (CF) are at risk of developing low bone mineral density (BMD) and fragility fractures. This paper presents consensus statements that summarise current knowledge of the epidemiology and pathophysiology of CF-related skeletal deficits and provides guidance on its assessment, prevention and treatment. The statements were validated using a modified Delphi methodology.

Extended valganciclovir prophylaxis to prevent cytomegalovirus after lung transplantation: a randomized, controlled trial.

Background: Cytomegalovirus (CMV) is the most prevalent opportunistic infection after lung transplantation. Current strategies do not prevent CMV in most at-risk patients.

Objective: To determine whether extending prophylaxis with oral valganciclovir from the standard 3 months to 12 months after lung transplantation is efficacious.

Managing complications following lung transplantation.

Lung transplantation has become a proven therapeutic option for patients with end-stage lung disease, extending life and providing improved quality of life to those who otherwise would continue to be breathless and oxygen-dependent. Over the past 20 years, considerable experience has been gained in understanding the multitude of medical and surgical issues that impact upon patient survival. Today, clinicians have an armamentarium of tools to manage diverse problems such as primary graft dysfunction, acute and chronic allograft rejection, airway anastomotic issues, infectious complications, renal dysfunction, diabetes and osteoporosis, hematological and gastrointestinal problems, malignancy, and other unique issues that confront immunosuppressed solid organ transplant recipients.

Vitamin d deficiency in cystic fibrosis.

Cystic Fibrosis is the most common inherited genetic respiratory disorder in the Western World. Hypovitaminosis D is almost universal in CF patients, likely due to a combination of inadequate absorption, impaired metabolism, and lack of sun exposure. Inadequate levels are associated with the high prevalence of bone disease or osteoporosis in CF patients, which is associated with increased morbidity including fractures, kyphosis, and worsening pulmonary status.

'Old' bones in young bodies: the tale of cystic fibrosis.

Purpose Of Review: Cystic fibrosis (CF) is the most common genetic disease within the white population and leads to premature respiratory failure. Approximately, 60 000 individuals are currently living with CF in North America and Europe, almost half of whom are adults.

Recent Findings: Dozens of studies across the globe indicate that CF adults have low bone density and increased rates of fractures.

Genetics of cystic fibrosis.

Cystic fibrosis (CF) is a complicated disease involving many organ systems. Identification of the cystic fibrosis transmembrane regulator (CFTR) genetic code has not only enhanced our understanding of the mechanism of CF pathology but has also provided explanations for phenotypic variation. Additionally, genetic testing has refined our ability to identify patients with CF and CF-related illnesses.

Update on cystic fibrosis-related bone disease: a special focus on children.

A high prevalence of low bone mineralization is documented in adult patients with cystic fibrosis (CF). Osteopenia is present in up to 85% of adult patients and osteoporosis in 10% to 34%. In children, study results are discordant probably because of comparisons to different control populations and corrections for bone size in growing children.

Post-transplantation lymphoproliferative disease: Epstein-Barr virus DNA levels, HLA-A3, and survival.

Rationale: Elevation in Epstein-Barr virus (EBV) circulating DNA has been proposed as a marker for development of post-transplant lymphoproliferative disease (PTLD), but few published data exist in the study of lung-transplant recipients.

Objectives: To determine if elevated EBV DNA levels, in combination with other risk factors, were predictive of PTLD.

Methods: We conducted a retrospective, single-center study examining all lung transplant recipients (n = 296) and EBV DNA levels (n = 612) using real-time TaqMan polymerase chain reaction.

Burkholderia gladioli: five year experience in a cystic fibrosis and lung transplantation center.

Background: The impact of infection with Burkholderia gladioli in cystic fibrosis, other chronic airway diseases and immunosuppressed patients is unknown.

Methods: A six-year retrospective review of all patients with B. gladioli infection was performed in a tertiary referral center with cystic fibrosis and lung transplantation programs.

High incidence of gastric bezoars in cystic fibrosis patients after lung transplantation.

Background: Bezoars are concretions of ingested matter which accumulate in the gastrointestinal tract and manifest as symptomatic foreign bodies. The aim of this study is to evaluate the incidence of gastric bezoars after lung transplantation and identify associated risk factors.

Methods: We performed a retrospective analysis of patients who underwent lung transplantation from December, 1992 through July, 2005 at our tertiary care medical center.

Everolimus in pulmonary transplantation: pharmacokinetics and exposure-response relationships.

Background: In this study we evaluated exposure, safety and efficacy data from an international trial of everolimus. We sought to identify a tolerated and efficacious range for blood levels of this agent in maintenance lung transplant recipients.

Methods: In a randomized, double-blind, multicenter trial, 213 maintenance lung transplant recipients received either everolimus 1.

Calcineurin inhibitor effects on growth and phenotype of human airway epithelial cells in vitro.

Calcineurin inhibitors (CIs) cyclosporin and tacrolimus form the basis for immunosuppression in lung transplantation, yet also exert biological effects on nonlymphoid tissue. With the advent of inhaled cyclosporin, we hypothesize that the airway epithelium is also subject to CI effects at high doses. The aim of this study was to identify human tracheobronchial epithelial cell (hTBEC) calcineurin gene expression and quantify effects of CIs on hTBEC growth, interleukin-1-beta stimulated IL-8 production and hTBEC phenotype.

C-C chemokine receptor 5 on stromal cells promotes pulmonary metastasis.

We have shown that mice that express the C-C chemokine receptor 5 (CCR5) have enhanced local tumor growth and an impaired response to vaccine therapy compared with CCR5 knockout (CCR5(-/-)) mice. Here, we extend these observations to evaluate the function of CCR5 in pulmonary metastasis and the mechanism underlying the diminished tumor growth in CCR5(-/-) mice. Lung metastases were counted in wild-type (WT) and CCR5(-/-) mice following the injection of 1 x 10(6) B16-F10 melanoma cells.