Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Diagnosis, and Management.

Chronic thromboembolic pulmonary hypertension (CTEPH), classified as World Health Organization (WHO) group 4 pulmonary hypertension (PH), is an interesting and rare pulmonary vascular disorder secondary to mechanical obstruction of the pulmonary vasculature from thromboembolism resulting in PH. The pathophysiology is complex, beginning with mechanical obstruction of the pulmonary arteries, which eventually leads to arteriopathic changes and vascular remodeling in the nonoccluded arteries and in the distal segments of the occluded arteries mediated by thrombus nonresolution, abnormal angiogenesis, endothelial dysfunction, and various local growth factors. Based on available data, CTEPH is a rare disease entity occurring in a small proportion (0.

Congenital and infantile malignant melanoma of the scalp: A systematic review.

Congenital and infantile malignant melanomas are rare and typically carry poor prognosis. The purpose of this article was to review the data on congenital and infantile malignant melanomas of the scalp in order to understand its presentation, diagnosis, management, and outcomes of congenital melanoma of scalp. We searched PubMed, CINAHL and Cochrane databases.

Cardiac Resynchronization Therapy for Heart Failure.

Cardiac resynchronization therapy (CRT) has emerged as a valued nonpharmacologic therapy in patients with heart failure, reduced ejection fraction (EF), and ventricular dyssynchrony manifest as left bundle branch block. The mechanisms of benefit include remodeling of the left ventricle leading to decreased dimensions and increased EF, as well as a decrease in the severity of mitral regurgitation. This article reviews the rationale, effects, and indications for CRT, and discusses the patient characteristics that predict response and considerations for nonresponders.

Advances in chemical pharmacotherapy for managing acute decompensated heart failure.

Acute decompensated heart failure (ADHF) contributes largely to the burden of heart failure and is associated with a poorer prognosis. Although numerous clinical trials evaluated the benefit of newer medications for ADHF, most of them were not successful. Areas covered: This review focusses on the updates on recent developments in chemical pharmacotherapy for the management of ADHF.

Ambulatory pulmonary artery pressure monitoring in advanced heart failure patients.

Heart failure (HF) is an emerging epidemic associate with significant morbidity, mortality, and health care expenditure. Although there were major advances in pharmacologic and device based therapies for the management of HF, mortality of this condition remains high. Accurate monitoring of HF patients for exacerbations is very important to reduce recurrent hospitalizations and its associated complications.

The association between socioeconomic status and the symptoms at diagnosis of celiac disease: a retrospective cohort study.

Background: There are little data on patient factors that impact diagnosis rates of celiac disease. This study aims to evaluate the association between patient socioeconomic status and the symptoms at diagnosis of celiac disease.

Methods: A total of 872 patients with biopsy-proven celiac disease were categorized based on the presence or absence of (1) diarrhea and (2) any gastrointestinal symptoms at diagnosis.

Update on pulmonary arterial hypertension pharmacotherapy.

Pulmonary artery hypertension (PAH) refers to several subgroups of disease in which the mean pulmonary artery pressure (mPAP) is elevated to more than 25 mm Hg, pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg, and an elevated pulmonary vascular resistance (PVR) > 3 Wood units as confirmed by right heart catheterization. The prevalence and geographic distribution of PAH vary depending on the type and etiology of the disease. Despite enormous efforts in the research and development of therapeutic agents in the last twenty years, the disease remains relatively incurable and the overall prognosis remains guarded.

Use of Inotropic Agents in Treatment of Systolic Heart Failure.

The most common use of inotropes is among hospitalized patients with acute decompensated heart failure, with reduced left ventricular ejection fraction and with signs of end-organ dysfunction in the setting of a low cardiac output. Inotropes can be used in patients with severe systolic heart failure awaiting heart transplant to maintain hemodynamic stability or as a bridge to decision. In cases where patients are unable to be weaned off inotropes, these agents can be used until a definite or escalated supportive therapy is planned, which can include coronary revascularization or mechanical circulatory support (intra-aortic balloon pump, extracorporeal membrane oxygenation, impella, left ventricular assist device, etc.

Use of Extracorporeal Membrane Oxygenation in Refractory Cardiogenic Shock.

Cardiogenic shock is a condition marked by low cardiac output and end-organ hypoperfusion frequently requiring hemodynamic support, and it carries a high mortality. Extracorporeal membrane oxygenation (ECMO) is an effective tool in providing mechanical circulatory support in patients with cardiogenic shock refractory to conventional medical therapies. ECMO can be an essential bridge to recovery, ventricular assist device implantation or transplant.

Socioeconomic Risk Factors for Celiac Disease Burden and Symptoms.

Background & Aims: Celiac disease (CD) affects approximately 1% of the population and negatively affects aspects of life including physical and social function. The relationship between socioeconomic (SE) factors, symptom severity, and perceived burden of living with CD is not well understood. The objective of this study was to assess the relationships between income, symptoms, and perceived burden of CD.

Patient perception of treatment burden is high in celiac disease compared with other common conditions.

Objectives: The only treatment for celiac disease (CD) is life-long adherence to a gluten-free diet (GFD). Noncompliance is associated with signs and symptoms of CD, yet long-term adherence rates are poor. It is not known how the burden of the GFD compares with other medical treatments, and there are limited data on the socioeconomic factors influencing treatment adherence.

Clinical utility of celiac disease-associated HLA testing.

Background: Negative predictive value (NPV) of celiac disease (CD)-related human leukocyte antigens (HLA) DQ2 and DQ8 approaches 100 % in individual patients. However, studies evaluating its exclusionary utility in patient groups are lacking.

Aim: We aim to assess the performance of HLA testing when applied to patient groups with varying characteristics and propose evidence-based recommendations for its clinical use.

Open conformation tissue transglutaminase testing for celiac dietary assessment.

Objectives: Anti tissue-transglutaminase antibody is the mainstay of celiac disease serologic testing. Whilst it has high sensitivity in patients on an unrestricted diet, sensitivity is poor for evaluation of gluten free diet adherence.

Aim: To assess the utility of a novel assay measuring Immunoglobulin-A antibodies to catalytically active open conformation tissue-transglutaminase in assessment of ongoing gluten exposure in celiac disease patients on an alleged gluten free diet.

The incidence and clinical spectrum of refractory celiac disease in a north american referral center.

Objectives: Refractory celiac disease (RCD) is one of the most serious causes of persistent symptoms in patients with celiac disease (CD). Published reports suggest that approximately half of patients in Europe are RCD type II, which carries a poor prognosis with a 5-year survival rate of ~50% compared with ~90% for RCD type I. However, disease patterns may be different in North America.