New concepts in palliative care in the intensive care unit.

Some patients admitted to an intensive care unit may face a terminal illness situation, which usually leads to death. Knowledge of palliative care is strongly recommended for the health care providers who are taking care of these patients. In many situations, the patients should be evaluated daily as the introduction of further treatments may not be beneficial to them.

Localization of Burkholderia cepacia complex bacteria in cystic fibrosis lungs and interactions with Pseudomonas aeruginosa in hypoxic mucus.

The localization of Burkholderia cepacia complex (Bcc) bacteria in cystic fibrosis (CF) lungs, alone or during coinfection with Pseudomonas aeruginosa, is poorly understood. We performed immunohistochemistry for Bcc and P. aeruginosa bacteria on 21 coinfected or singly infected CF lungs obtained at transplantation or autopsy.

Persistent Left Superior Vena Cava Identified During Central Line Placement: A Case Report.

INTRODUCTION: A persistent left superior vena cava is found in 0.3-0.5% of the general population and in up to 10% of patients with a congenital cardiac anomaly.

Effects of lung transplantation on inpatient end of life care in cystic fibrosis.

Background: The impact of lung transplantation on end of life care in cystic fibrosis (CF) has not been widely investigated.

Methods: Information about end of life care was collected from records of all patients who died in our hospital from complications of CF between 1995 and 2005. Transplant and non-transplant patients were compared.

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.

Classifying severity of cystic fibrosis lung disease using longitudinal pulmonary function data.

Rationale: The study of genetic modifiers in cystic fibrosis (CF) lung disease requires rigorous phenotyping. One type of genetic association study design compares polymorphisms in patients at extremes of phenotype, requiring accurate classification of pulmonary disease at varying ages.

Objective: To evaluate approaches to quantify severity of pulmonary disease and their ability to discriminate between patients with CF at the extremes of phenotype.

Genetic modifiers of lung disease in cystic fibrosis.

Background: Polymorphisms in genes other than the cystic fibrosis transmembrane conductance regulator (CFTR) gene may modify the severity of pulmonary disease in patients with cystic fibrosis.

Methods: We performed two studies with different patient samples. We first tested 808 patients who were homozygous for the DeltaF508 mutation and were classified as having either severe or mild lung disease, as defined by the lowest or highest quartile of forced expiratory volume in one second (FEV1), respectively, for age.

Massive hemoptysis in cystic fibrosis.

Background: Massive hemoptysis is a complication commonly reported in patients with cystic fibrosis (CF). An understanding of the pathophysiology of this complication and its consequences is important for the management of patients with CF.

Objectives: To identify risk factors associated with massive hemoptysis, and to determine the prognosis of patients following an episode of massive hemoptysis.

Restoration of the cystic fibrosis transmembrane conductance regulator function by splicing modulation.

A significant fraction of disease-causing mutations affects pre-mRNA splicing. These mutations can generate both aberrant and correct transcripts, the level of which varies among different patients. An inverse correlation was found between this level and disease severity, suggesting a role for splicing regulation as a genetic modifier.

Airway retention of materials of different solubility following local intrabronchial deposition in dogs.

We used a gamma camera to monitor the retention and clearance of radiolabeled human serum albumin (HSA), a water-soluble material with molecular weight of 66,000 Daltons, and radiolabeled sulfur colloid (SC), an insoluble submicron (0.22 microm) particle, following localized deposition in a 4-5-mm bronchus in each of five dogs. The average retention time of HSA was significantly greater than that of SC both in the deposition site (23.

Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients.

Current theories of CF pathogenesis predict different predisposing "local environmental" conditions and sites of bacterial infection within CF airways. Here we show that, in CF patients with established lung disease, Pseudomonas aeruginosa was located within hypoxic mucopurulent masses in airway lumens. In vitro studies revealed that CF-specific increases in epithelial O(2) consumption, linked to increased airway surface liquid (ASL) volume absorption and mucus stasis, generated steep hypoxic gradients within thickened mucus on CF epithelial surfaces prior to infection.