Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia.

Description: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications.

Co-presentation of pulmonary arteriovenous malformation (PAVM) and multifocal benign metastasizing leiomyoma (BML) of lungs: Diagnostic dilemma and successful endovascular treatment.

A 50-year-old female with a 20-year history of multifocal pulmonary benign metastasizing leiomyoma (BML), and asthma presented with subacute worsening of chronic dyspnea. A contrast-enhanced computerized tomography of the chest showed a single 1.4 × 1.

Characterization of a family mutation in the 5' untranslated region of the endoglin gene causative of hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) is a vascular disease characterized by nose and gastrointestinal bleeding, telangiectases in skin and mucosa, and arteriovenous malformations in major internal organs. Most patients carry a mutation in the coding region of the endoglin (ENG) or activin A receptor type II-1 (ACVRL1) gene. Nonetheless, in around 15% of patients, sequencing analysis and duplication/deletion tests fail to pinpoint mutations in the coding regions of these genes.

Prevalence of left heart contrast in healthy, young, asymptomatic humans at rest breathing room air.

Our purpose was to report the prevalence of healthy, young, asymptomatic humans who demonstrate left heart contrast at rest, breathing room air. We evaluated 176 subjects (18-41 years old) using transthoracic saline contrast echocardiography. Left heart contrast appearing ≤3 cardiac cycles, consistent with a patent foramen ovale (PFO), was detected in 67 (38%) subjects.

Communication by nurses in the intensive care unit: qualitative analysis of domains of patient-centered care.

Background: High-quality communication is a key determinant and facilitator of patient-centered care. Nurses engage in most of the communication with patients and patients' families in the intensive care unit.

Objective: To perform a qualitative analysis of nurses' communications.

Pathogenetic and predictive value of biomarkers in patients with ALI and lower severity of illness: results from two clinical trials.

Plasma and bronchoalveolar lavage (BAL) biomarkers related to the pathogenesis of acute lung injury (ALI) have previously been associated with poorer clinical outcomes and increased disease severity among patients with ALI. Whether these biomarkers have predictive value in a less severely ill population that excludes septic patients with high APACHE II scores is currently unknown. We tested the association of plasma and BAL biomarkers with physiological markers of ALI severity or clinically relevant outcomes in a secondary analysis of a clinical trial of activated protein C for the treatment of ALI.

Life-sustaining treatment decisions in the ICU for patients with ESLD: a prospective investigation.

We conducted a prospective study in the ICU of life-sustaining treatment and comfort care decisions over time in patients with end-stage liver disease (ESLD) from the perspectives of patients, family members, and healthcare professionals. Six patients with ESLD, 19 family members, and 122 professionals participated. The overarching theme describing the decision-making process was "on the train.

Randomized clinical trial of activated protein C for the treatment of acute lung injury.

Rationale: Microvascular injury, inflammation, and coagulation play critical roles in the pathogenesis of acute lung injury (ALI). Plasma protein C levels are decreased in patients with acute lung injury and are associated with higher mortality and fewer ventilator-free days.

Objectives: To test the efficacy of activated protein C (APC) as a therapy for patients with ALI.

Extravascular lung water in sepsis-associated acute respiratory distress syndrome: indexing with predicted body weight improves correlation with severity of illness and survival.

Objectives: To determine whether extravascular lung water predicts survival in patients with early acute respiratory distress syndrome, to determine the relationship between extravascular lung water and other markers of lung injury, and to examine if indexing extravascular lung water with predicted body weight (EVLWp) strengthens its discriminative power.

Design: Extravascular lung water and other markers of lung injury were measured prospectively in 19 patients with sepsis-induced acute respiratory distress syndrome for 3 days.

Setting: The intensive care units of an academic tertiary referral hospital.

Extraction of dental crowns from the airway: a multidisciplinary approach.

We describe two cases of airway foreign bodies (FB) consisting of a dental crown. The shape and composition of dental crowns complicate their extraction from the tracheobronchial tree, sometimes necessitating thoracotomy. We describe the use of a multidisciplinary approach involving rigid and flexible bronchoscopy in concert with the use of wire snares under fluoroscopic guidance for extraction of these challenging FB.

Management of asthma exacerbations.

The 1997 Expert Panel Report 2 from the National Asthma Education and Prevention Program* details principles and goals for managing asthma exacerbations, based on scientific literature and the opinion of the panel. The panel's recommendations are summarized here, along with approaches to the evaluation and management of patients with asthma exacerbations. Methods to assess and classify the severity of asthma exacerbations are discussed, and treatment objectives for mild, moderate, and severe exacerbations are presented, along with a discussion of postinfectious acute airway hyperresponsiveness.