Mortality Risk and Pulmonary Function in Adults With Cystic Fibrosis at Time of Wait Listing for Lung Transplantation.

Background: Lung transplantation (LTx) benefit for survival in cystic fibrosis (CF) patients placed on the wait list is not well studied.

Methods: To predict the relationship between initial forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) and the hazard ratio (HR) associated with LTx in CF patients, the United Network for Organ Sharing database was queried from 2005 to 2006 for adult patients with CF. Survival was assessed from wait list entry time until death on wait list, death after LTx, or censoring.

Age-based analysis of pediatric upper airway dimensions using computed tomography imaging.

Background: Recent studies have challenged the historically accepted fact that the larynx is cone-shaped in infants and children. The present study used computed tomography (CT)-based measurements to evaluate airway dimensions. The purpose of this investigation was to determine the dimensional transition between the subglottic area and the cricoid ring in children.

Eucapnic Voluntary Hyperventilation to Detect Exercise-Induced Bronchoconstriction in Cystic Fibrosis.

Background: Exercise-induced bronchoconstriction (EIB) has not been well studied in cystic fibrosis (CF), and eucapnic voluntary hyperventilation (EVH) testing has not been used as an objective assessment of EIB in CF to date.

Methods: A prospective cohort pilot study was completed where standard EVH testing was completed by 10 CF patients with forced expiratory volume in 1 s (FEV1) ≥70% of predicted. All patients also completed a cardiopulmonary exercise test (CPET) with pre- and post-CPET spirometry as a comparative method of detecting EIB.

Geographic variations in cystic fibrosis: An analysis of the U.S. CF Foundation Registry.

Background: Emerging evidence suggests that the prevalence of pathogens common in cystic fibrosis (CF) may be unevenly distributed across the United States (U.S.).

Influence of pulmonary hypertension on survival in advanced lung disease.

Background: Effects of pulmonary hypertension (PH) in advanced lung disease remain unclear.

Methods: The United Network for Organ Sharing database was queried from 1987 to 2013 to assess survival lung transplant candidates to determine influence of PH in advanced lung disease. Thresholds included mean pulmonary artery pressure ≥ 25 mmHg (mild PH) and 35 mmHg (severe PH).

Changes in Pulmonary Function and Controlled Ventilation-High Resolution CT of Chest After Antibiotic Therapy in Infants and Young Children with Cystic Fibrosis.

Background: Infants with cystic fibrosis (CF) develop early progressive lung disease which may be asymptomatic. Infant pulmonary function tests (IPFT) and controlled ventilation-high resolution computed tomography (CV-HRCT) of chest can detect early asymptomatic lung disease. It is not well established that these objective measures can detect changes in lung disease after clinical interventions.

Method of isolated ex vivo lung perfusion in a rat model: lessons learned from developing a rat EVLP program.

The number of acceptable donor lungs available for lung transplantation is severely limited due to poor quality. Ex-Vivo Lung Perfusion (EVLP) has allowed lung transplantation in humans to become more readily available by enabling the ability to assess organs and expand the donor pool. As this technology expands and improves, the ability to potentially evaluate and improve the quality of substandard lungs prior to transplant is a critical need.

Outcomes in pediatric lung transplant recipients receiving adult allografts.

Background: Clinical practice in the United States has no restrictions in allocating lungs from adult donors to pediatric recipients.

Methods: The United Network for Organ Sharing database was queried from 1987 to 2013 for pediatric lung transplant recipients (aged less than 18 years) to assess survival using continuous donor age in years and two donor age groups, ≥ 18 years and > 30 years, for analysis.

Results: Of 930 pediatric lung transplants, basic survival analysis identified a mortality hazard when adult lung allografts were transplanted into pediatric recipients; however, multivariate Cox models demonstrated that continuous donor age (hazard ratio [HR] 1.

Improvement of hepatic steatosis in cystic fibrosis with ivacaftor therapy.

Treatment of liver disease, including hepatic steatosis, in patients with cystic fibrosis (CF) is limited. With the development of ivacaftor, which corrects the gating defect of the CF transmembrane regulator channel, there is a potential new therapy available for this subgroup of the CF patient population. We present an adolescent with CF who had significant improvement in hepatic steatosis with ivacaftor treatment while hypothesizing on a mechanism of why it occurred.

Influence of age on survival in adult patients on extracorporeal membrane oxygenation before lung transplantation.

Background: Extracorporeal membrane oxygenation (ECMO) is widely accepted as a means of support before lung transplantation (LTx), but limited data exist regarding its impact on long-term survival in association with patient age and covariates that may be influencing clinical outcomes.

Methods: The United Network for Organ Sharing (UNOS) database was queried from 2000 to 2013 for adult recipients (≥18 years) to assess the impact of age on survival in patients on ECMO before LTx.

Results: From a total 17,556 adult lung recipients, 17,441 were included for univariate and Kaplan-Meier function analysis, 15,553 for multivariate Cox models and 364 for propensity score matching.

Inhalable nanoparticulate powders for respiratory delivery.

Unlabelled: Nanoparticles are extensively studied for drug delivery and are proving to be effective in drug delivery and the diagnostic field. Drug delivery to lungs has its advantages over other routes of administration. Inhalable powders consisting of nanoparticles are gaining much interest in respiratory research and clinical therapy.

Plasma exchange on venovenous extracorporeal membrane oxygenation with bivalirudin anticoagulation.

A pediatric patient requiring venovenous (VV) extracorporeal membrane oxygenation (ECMO) as a bridge to lung transplantation developed heparin-induced thrombocytopenia. Unfractionated heparin was discontinued, and a bivalirudin infusion was started. During the lung transplant evaluation, he was found to have allosensitization, requiring treatment with plasma exchange along with pulse methylprednisolone, rituximab, bortezomib, and pooled immunoglobulin infusion.

Improving accessibility to lung transplantation for children through air transport.

Introduction: There are a limited number of pediatric lung transplant programs in the United States; therefore, geographical barriers limit critically ill children who may be good candidates for lung transplantation.

Materials And Methods: With advancements in technology, extracorporeal membrane oxygenation (ECMO) is becoming an option to bridge children requiring advanced life support to lung transplantation and offers the opportunity to transport critically ill children to pediatric lung transplant centers.

Results: Air transport services with rotor-wing and fixed-wing aircraft were successfully developed and quickly became as a vital component of our pediatric lung transplant program offering ECMO for both transport and as a bridge to lung transplantation to children throughout the United States.

Effect of pulmonary hypertension on survival in patients with idiopathic pulmonary fibrosis after lung transplantation: an analysis of the United Network of Organ Sharing registry.

Background: Pulmonary hypertension (PH) is a comorbidity associated with idiopathic pulmonary fibrosis (IPF). There is limited research regarding the effect on survival after lung transplantation (LTx).

Methods: To assess the effect of PH on survival in patients with IPF who received LTx, the United Network for Organ Sharing was queried for eligible patients with recorded mean (PAmean) and systolic (PAsystolic) pulmonary artery pressure.

High-risk age window for mortality in children with cystic fibrosis after lung transplantation.

LTx in children with CF remains controversial. The UNOS database was queried from 1987 to 2013 for CF patients <18 yr of age at time of transplant. PCHR model was used to quantify hazard of mortality.

Survival in children on extracorporeal membrane oxygenation at the time of lung transplantation.

Limited data exist on ECMO at the time of LTx in children. The UNOS database was queried from 2000 to 2013 for pediatric lung transplant recipients (<18 yr) to assess post-transplant survival of patients on ECMO at the time of LTx. Of 587 pediatric recipients with 17 on ECMO, 585 were used for univariate and Kaplan-Meier function analysis, 535 for multivariate Cox models, and 24 for propensity score matching.

Dry powder inhalers in COPD, lung inflammation and pulmonary infections.

Introduction: The number of pulmonary diseases that are effectively treated by aerosolized medicine continues to grow.

Areas Covered: These diseases include chronic obstructive pulmonary disease (COPD), lung inflammatory diseases (e.g.

Influence of donor and recipient age in lung transplantation.

Background: No restrictions exist for allocating donor lungs according to age in the United States.

Methods: A retrospective cohort study was performed of lung transplant recipients from 1987 to 2013 through a query of the United Network for Organ Sharing (UNOS) thoracic database. Age thresholds used were recipients (R) aged 60 years (R60 group) and 65 years (R65 group) and donors aged 50 years; each sub-group was analyzed, totaling 8 groups.

Impact of induction immunosuppression on survival in heart transplant recipients: a contemporary analysis of agents.

Introduction: The impact of induction immunosuppression on long-term survival in heart transplant recipients is unclear. Over the past three decades, practices have varied as induction agents have changed and experiences grew. We sought to evaluate the effect of contemporary induction immunosuppression agents in heart transplant recipients with the primary endpoint of survival, utilizing national registry data.

Obstructive sleep apnea-hypopnea results in significant variations in cerebral hemodynamics detected by diffuse optical spectroscopies.

The objective of this study was to adapt a novel near-infrared diffuse correlation spectroscopy (DCS) flow-oximeter for simultaneous and continuous monitoring of relative changes in cerebral blood flow (rCBF) and cerebral oxygenation (i.e. oxygenated/deoxygenated/total hemoglobin concentration: Δ[HbO2]/Δ[Hb]/ΔTHC) during overnight nocturnal polysomnography (NPSG) diagnostic test for obstructive sleep apnea-hypopnea (OSAH).

Pulmonary hypertension in cystic fibrosis with advanced lung disease.

Rationale: The impact of pulmonary hypertension (PH) on survival in cystic fibrosis (CF) remains unclear.

Objectives: To determine the influence of PH on survival in the CF population.

Methods: The United Network for Organ Sharing database was queried from 1987 to 2013 to identify first-time lung transplant candidates who were tracked from wait list entry date until death or censoring to determine influence of PH.