Cross-sectional validation study of patient-reported outcomes in patients with paroxysmal nocturnal haemoglobinuria.

Background: Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, acquired, clonal haemopoietic stem cell disorder that causes chronic intravascular haemolysis, increases the risk of thrombosis and results in significant patient morbidity and mortality. The symptoms of PNH may have a major impact on patient quality of life.

Aims: To assess patient fatigue and health-related quality of life in 29 patients with PNH using the Functional Assessment of Chronic Illness Therapy Fatigue subscale version 4 (FACIT-Fatigue) and the European Organization for Research and Treatment of Cancer Quality-of-Life Questionnaire-C30, version 3 (EORTC QLQ-C30).

The evidence for disease modification in Parkinson's disease.

Disease modification or slowing the progression of any neurodegenerative disorder represents a dire unmet need. There have been trials for several decades specifically designed to help evaluate whether a specific therapy might be able to slow the progression of Parkinson's disease (PD) or be disease modifying. Trials evaluating the use of coenzyme Q10, pramipexole, and levodopa suggest that these medications offer symptomatic benefit uniquely, while other studies reveal that rasagiline and selegiline may be disease modifying.

The use of the StarClose device for obtaining femoral artery hemostasis.

Background: Following percutaneous intervention, obtaining femoral artery hemostasis quickly and reliably with few complications is of considerable importance. While the StarClose device has been studied in patients undergoing cardiac catheterization, its use in patients undergoing treatment for peripheral vascular disease has rarely been reported. It is the purpose of this report to determine whether the Starclose is able to deliver safe and effective femoral artery closure in this group of patients.

Leber's Hereditary Optic Neuropathy.

Leber's hereditary optic neuropathy (LHON) is a maternally inherited blinding disease with variable penetrance. Three primary mitochondrial DNA mutations, affecting the respiratory complex I, are necessary but not sufficient to cause blindness. Reduced efficiency of ATP synthesis and increased oxidative stress are believed to sensitize the retinal ganglion cells to apoptosis.

Emergency department ultrasound by nurse practitioners.

Purpose: Bedside ultrasound (u/s) examinations by emergency physicians (EPs) have been integrated into clinical emergency medicine (EM) training programs and the efficacy and success of an introductory u/s training course, based on established guidelines, has been previously described. We used this same course to teach EM nurse practitioners (NPs) bedside u/s and then assessed the adequacy of NP ultrasonography 1 year following the course.

Data Sources: Five NPs were included in a standardized 16-h EM u/s training program.

The utility of serial peak flow measurements in the acute asthmatic being treated in the ED.

Background: Peak flow is used extensively in emergency departments (EDs) to both assess asthma patient's status on arrival as well as to document clinical improvement during treatment. Many algorithms suggest serial peak expiratory flow (PEF) measurements during an ED stay.

Objective: The aim of the study was to assess the contribution of serial PEF in describing the overall improvement of asthmatics over the course of an ED visit for acute exacerbation of their asthma.

Racial/ethnic differences in bystander CPR in Los Angeles, California.

Background: Bystander CPR (BCPR) has been demonstrated to improve rates of return of spontaneous circulation, survival to hospital admission, and quality of life in survivors. While previous studies have shown that African Americans are less likely to receive BCPR than Caucasians even after adjusting for variables such as socioeconomic status, BCPR rates in Latinos have not been reported.

Objective: To describe BCPR rates in an urban African American and Latino population as compared to Caucasians.

Treatment of chest wall implosion injuries without thoracotomy: technique and clinical outcomes.

Background: Chest wall implosion injuries secondary to side impact are unusual but devastating injuries. The purpose of this series is to describe the clinical entity, present a surgical technique to reduce and repair the thoracic cage deformity without thoracotomy, and report outcomes in nine patients.

Study: Institutional review board approved retrospective case series, surgical technique.

Does reflux in orthotopic diversion matter? A randomized prospective comparison of the Studer and T-pouch ileal neobladders.

Purpose: Orthotopic neobladder reconstruction has become a standard form of urinary diversion in many centers for patients undergoing radical cystectomy for bladder cancer. There is still controversy about the best technique for construction of the neobladder, and especially whether it is necessary to include an antireflux mechanism.

Methods: We designed a prospective randomized clinical trial comparing two forms of ileal neobladder: the Studer pouch and the T-pouch.

Measurement of ultraviolet exposure in epidemiological studies of skin and skin cancers.

As our understanding of the role of UV in causing skin cancer continues to expand, researchers and clinicians must continue to remain up to date on the various means by which UV exposure can be quantified. The purpose of this article was to review the current methods used to measure lifetime exposure to UV and to summarize the strengths and weaknesses of each of these approaches. Thus we include here a review of research articles that deal with questionnaire reliability and physical examination.

The presence of an Emergency Airway Response Team and its effects on in-hospital Code Blue.

The survival rate from in-hospital cardiac arrest due to pulseless electrical activity (PEA)/asystole in our institution was higher than expected (70%). It was the impression of the Emergency Department-led Code Blue Team (CBT) that many of these patients were actually suffering respiratory arrests before their cardiac events. To address this, the facility developed an early intervention team focused on early airway intervention-the Emergency Airway Response Team (EART).

Ischemic mass effect from biliary surgical clips.

Migrating surgical clips in the hepatic hilum are known causes of biliary stricture or obstruction, most often due to direct intraluminal obstruction or secondary stone formation. Two cases are reported on patients with previous cholecystectomies presenting with delayed symptoms of biliary tract stricture. Both patients were successfully treated with a resection of the strictured area and a Roux-en-Y hepatico-jejunostomy.

Subclinical carriers and conversions in Leber hereditary optic neuropathy: a prospective psychophysical study.

Purpose: The authors previously presented the results of their 2001 field investigation to rural Brazil to investigate a 336-member pedigree of Leber hereditary optic neuropathy (LHON). The present work describes the yearly field investigations 2001 to 2005, utilizing a variety of highly sophisticated psychophysical and electrophysiologic procedures, in asymptomatic LHON carriers, some of whom converted to affected status.

Methods: Careful, repeated examinations of 75 carriers of homoplasmic 11778 LHON mtDNA J-haplogroup mutants were performed as part of the field investigation of this pedigree.

The use of bedside ultrasound in cardiac arrest.

Background: The decision to terminate resuscitative measures in the setting of cardiac arrest is based on several criteria, some of which are subjective. Ultrasound in the emergency department has potentially added an objective data point to assist in this decision.

Objective: We sought to determine if emergency physicians who were trained in the use of ultrasound use it in cardiac arrest scenarios and if so, what effect they believe it has on the duration of the resuscitative event.

Myth: Intravenous amiodarone is safe in patients with atrial fibrillation and Wolff-Parkinson-White syndrome in the emergency department.

Wolff-Parkinson-White (WPW) syndrome with atrial fibrillation (AF) is a potentially life-threatening problem requiring rapid conversion to sinus rhythm. The most recent American Heart Association guidelines for the treatment of patients with WPW, published in conjunction with the 2000 Advanced Cardiac Life Support (ACLS) guidelines, suggests that intravenous amiodarone is a first-line therapy for AF-WPW; however the evidence suggests this is a potentially dangerous myth.

Living related donor nephrectomy in transfusion refusing donors.

Many transplant programs are averse to evaluate potential kidney donors with preferences against accepting human blood products. We examined the donor and graft outcomes between our transfusion-consenting (TC) and transfusion-refusing (TR) live kidney donors to determine whether a functional or survival disadvantage resulted from the disallowance of blood product transfusion during live donor (LD) nephrectomy. From July, 1999 to August, 2005, 82 live donor nephrectomies were performed, eight of who were TR donors (10%).

Long-term safety and tolerability of oxcarbazepine in painful diabetic neuropathy.

Background: Painful diabetic neuropathy is a common complication of diabetes and often resistant to treatment with standard analgesics. Treatment of diabetic neuropathy with antiepileptic drugs may provide pain relief.

Aim: To evaluate the long-term safety and tolerability of oxcarbazepine in two studies investigating the treatment of diabetic neuropathy.

Myth: blood transfusion is effective for sickle cell anemia-associated priapism.

Objective: Priapism is a recognized complication of sickle cell anemia (SCA). When initial conventional treatments fail, simple or exchange blood transfusion has been advocated as a secondary intervention. However, recent literature suggests this may not be an effective therapy and may have significant neurologic sequelae.

A modern biomaterial for adhesion prevention.

A biomaterial composed of carboxymethylcellulose, poly(ethylene oxide), and calcium can be prepared in a variety of ways to reduce fibrin deposition and adhesion formation. This biomaterial platform can be formulated into a flowable gel with tissue adherence appropriate for use in minimally invasive surgery. The device remains at the site of placement even in gravitationally dependent areas.