Clinical Impact of Blood Culture Results in Acutely Ill Hospitalized Adult Patients With Cystic Fibrosis.

Background: Blood cultures are obtained clinically to confirm site and source of acute infection as well as to guide effective antibiotic therapies. Patients with cystic fibrosis (CF) are at risk for blood stream infection (BSI) as identified from positive blood culture results.

Methods: A retrospective chart review was performed of 190 adult CF patients from January 1, 2001 through December 1, 2015.

Characteristics of Resting Metabolic Rate in Critically Ill, Mechanically Ventilated Adults With Cystic Fibrosis.

Background: Critically ill patients with cystic fibrosis may be especially sensitive to the negative consequences of overfeeding and underfeeding, yet there is almost no information available about the energy needs of these patients. The purpose of this study was to characterize the metabolic rate of critically ill adult patients with cystic fibrosis requiring mechanical ventilation.

Methods: This was an observational study in which the resting metabolic rate, oxygen consumption, and carbon dioxide production of adult patients with cystic fibrosis requiring critical care, sedation, and mechanical ventilation were measured with indirect calorimetry.

Prediction equation to estimate dead space to tidal volume fraction correlates with mortality in critically ill patients.

Objective: The measurement of dead space to tidal volume fraction (Vd/Vt) using various methodologies has been shown to be a reliable predictor of mortality in critically ill patients. In this study, we evaluated the correlation of a validated equation using clinically available information to predict calculation of Vd/Vt with clinically relevant outcome parameters in patients requiring mechanical ventilation.

Methods: Calculations of Vd/Vt were obtained based upon a previously published prediction equation for dead space ventilation fraction: Vd/Vt = 0.

Severe hypercapnia in critically ill adult cystic fibrosis patients.

Background: Cystic fibrosis (CF) is a monogenetic autosomal recessive multi-organ disease affecting approximately 50,000 patients worldwide. Overall median survival is continually increasing but pulmonary disease remains the most common cause of death. Guidelines have been published in relation to the outpatient maintenance of lung health for CF patients and treatment of acute lung exacerbations but little information exists about the management of the critically ill CF patient.

Predicting dead space ventilation in critically ill patients using clinically available data.

Objective: To develop and validate an equation to predict dead space to tidal volume ratio (Vd/Vt) from clinically available data in critically ill mechanically ventilated patients.

Design: Prospective, observational study using a convenience sample of patients whose arterial blood gas and respiratory gas exchange had been measured with indirect calorimetry.

Setting: Medical and surgical critical care units of a university medical center.

Cystic fibrosis pulmonary guidelines: airway clearance therapies.

Cystic fibrosis (CF) is a genetic disease characterized by dehydration of airway surface liquid and impaired mucociliary clearance. As a result, there is difficulty clearing pathogens from the lung, and patients experience chronic pulmonary infections and inflammation. Clearance of airway secretions has been a primary therapy for those with CF, and a variety of airway clearance therapies (ACTs) have been developed.

Cystic fibrosis lung disease in adult patients.

As the longevity of all patients with cystic fibrosis (CF) continues to increase (median 2005 survival=36.8 years), more adult patients will be receiving their medical care from nonpediatric adult-care providers. Cystic fibrosis remains a fatal disease, with more than 80% of patients dying after the age of 18 years, and most deaths resulting from pulmonary disease.

Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health.

Rationale: Cystic fibrosis is a recessive genetic disease characterized by dehydration of the airway surface liquid and impaired mucociliary clearance. As a result, individuals with the disease have difficulty clearing pathogens from the lung and experience chronic pulmonary infections and inflammation. Death is usually a result of respiratory failure.

Respiratory systems abnormalities and clinical milestones for patients with amyotrophic lateral sclerosis with emphasis upon survival.

Respiratory system complications and abnormalities are common in patients with amyotrophic lateral sclerosis (ALS) and respiratory failure remains the most common cause of death. Extensive epidemiological longitudinal data have documented the extent, magnitude, and clinical course of these abnormalities, but few studies have provided objective information that can have prognostic significance for individual patients. In this study, the reported data represent results from a retrospective review of the medical records of 153 patients with ALS cared for at a single institution (The Penn State Milton S.

A clinical pilot study: high frequency chest wall oscillation airway clearance in patients with amyotrophic lateral sclerosis.

Respiratory complications are common in patients with amyotrophic lateral sclerosis (ALS) with respiratory failure representing the most common cause of death. Ineffective airway clearance resultant from deficient cough frequently contributes to these abnormalities. We sought to evaluate the effectiveness of high frequency chest wall oscillation (HFCWO) administered through the Vest Airway Clearance System when added to standard care in preventing pulmonary complications and prolonging the time to death in patients with ALS.