PubMed Journals Articles About "Paroxysmal Hemoglobinuria" RSS

00:15 EST 10th December 2018 | BioPortfolio

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Showing "Paroxysmal Hemoglobinuria" PubMed Articles 1–25 of 103

Ecthyma Gangrenosum in Paroxysmal Nocturnal Hemoglobinuria.

Correction to: Effects of eculizumab treatment on quality of life in patients with paroxysmal nocturnal hemoglobinuria in Japan.

In the original publication of this article, Tables 2, 3 and 4 were published incorrectly. The corrected tables 2, 3 and 4 are given in the following pages.

How we treat paroxysmal nocturnal hemoglobinuria: a consensus statement of the Canadian PNH Network and review of the national registry.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disease characterized by intravascular hemolysis, thrombophilia, and marrow failure. Its phenotype is due to absent or reduced expression of GPI-linked complement regulators and subsequent sensitivity of hematopoietic cells to complement-mediated damage and lysis. Introduction of the terminal complement inhibitor eculizumab drastically improved outcomes in PNH patients; however, despite this improvement, there remain several challenges faced by...

Evolutionary dynamics of paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal blood disorder characterized by hemolysis and a high risk of thrombosis, that is due to a deficiency in several cell surface proteins that prevent complement activation. Its origin has been traced to a somatic mutation in the PIG-A gene within hematopoietic stem cells (HSC). However, to date the question of how this mutant clone expands in size to contribute significantly to hematopoiesis remains under debate. One hypothesis posits the existenc...

Prothrombotic mechanisms in patients with congenital p.Cys89Tyr mutation in CD59.

Thrombosis is the prognostic factor with the greatest effect on survival in patients with paroxysmal nocturnal hemoglobinuria (PNH), who lack dozens of membrane surface proteins. We recently described a primary homozygous Cys89Tyr congenital nonfunctioning CD59 in humans with clinical manifestation in infancy, associated with chronic hemolysis, recurrent strokes, and relapsing peripheral demyelinating neuropathy. Here we investigated hypercoagulability mechanisms characterizing the syndrome.

Non-paroxysmal disorder in infant.

Non-epileptic paroxysmal disorders (PNED) are defined as events that mimic epileptic seizures. Its onset is usually sudden and short-lived, caused by brain dysfunction of various origins, but not due to excessive neuronal firing. Its incidence is higher than the epilepsy (10:1). They can occur at any age but are most common in children, especially in the first year of life. The immature nervous system in childhood causes in this period, paroxysmal manifestations that are very diverse and different from othe...

The Risk of Clonal Evolution of Granulocyte Colony-Stimulating Factor for Acquired Aplastic Anemia: A Systematic Review and Meta-Analysis.

This meta-analysis aimed to evaluate the risk of clonal evolution of granulocyte colony-stimulating factor (G-CSF) in acquired aplastic anemia (AA), and whether the use of G-CSF increases the occurrence of secondary malignant neoplasms, mainly myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) or paroxysmal nocturnal hemoglobinuria (PNH).

Eculizumab Bridging Before Bone Marrow Transplant for Marrow Failure Disorders is Safe and Does Not Limit Engraftment.

Paroxysmal nocturnal hemoglobinuria (PNH) often develops secondary to other bone marrow failure (BMF) disorders, especially aplastic anemia (AA). Patients with the AA/PNH syndrome may require treatment with both eculizumab to reduce intravascular hemolysis and the risk of thrombosis and allogeneic stem cell transplant for the severe BMF. There has been concern that eculizumab could adversely affect the outcomes for transplant in these patients.

Identification of paroxysmal atrial fibrillation subtypes in over 13,000 individuals.

Paroxysmal atrial fibrillation (PAF) is broadly defined despite high variability in the occurrence and duration of PAF episodes.

Non-epileptic paroxysmal disorders in pre-school children.

Paroxysmal events are commonly encountered in toddlers. These events include a variety of conditions with different manifestations and pathophysiology. For that reason, the diagnosis of these events can be challenging. In some instances, studies such as EEG and polysomnogram may be useful to differentiate between epileptic and non-epileptic events. In the majority of cases, a complete clinical history is enough to make an appropriate diagnosis. In this article, we review some of the most common paroxysmal n...

16p11.2 deletion in patients with paroxysmal kinesigenic dyskinesia but without intellectual disability.

Mutations of the PRRT2 gene are the most common cause for paroxysmal kinesigenic dyskinesia. However, patients with negative PRRT2 mutations are not rare. The aim of this study is to determine whether copy number variant of PRRT2 gene is another potential pathogenic mechanism in the patients with paroxysmal kinesigenic dyskinesia with negative PRRT2 point and frameshift mutations.

Complement activation by human red blood cell antibodies: hemolytic potential of antibodies and efficacy of complement inhibitors assessed by a sensitive flow cytometric assay.

Therapeutic intervention strategies in complement-mediated hemolytic diseases are still inappropriate, and lethal events cannot be reliably prevented. As an in vitro model of intravascular hemolysis, a sensitive flow cytometric assay was designed using red blood cells (RBCs) of patients with paroxysmal nocturnal hemoglobinuria (PNH) as target cells. Complement activation by human allo- and autoantibodies directed against RBC antigens and the effect of different complement inhibitors were studied.

Paroxysmal dyskinesias - disorder categories, their causes and treatment.

Paroxysmal dyskinesias refer to category of abnormal involuntary movements, such as chorea, dystonia, athetosis, ballism or their various configurations. Depending on the type of seizure, sudden movement, stress, emotions, coffee or alcohol may be the trigger factors. Acute seizures are characterized by short duration and are self-limitated. Patients present correct portray of movements between seizures. Intact consciousness during seizure is the invariable characteristic of all paroxysmal dyskinesias. The ...

Percutaneous versus thoracoscopic ablation of symptomatic paroxysmal atrial fibrillation: a randomised controlled trial - the FAST II study.

The most efficient first-time invasive treatment, for achieving sinus rhythm, in symptomatic paroxysmal atrial fibrillation has not been established. We aimed to compare percutaneous catheter and video-assisted thoracoscopic pulmonary vein radiofrequency ablation in patients referred for first-time invasive treatment due to symptomatic paroxysmal atrial fibrillation. The primary outcome of interest was the prevalence of atrial fibrillation with and without anti-arrhythmic drugs at 12 months.

A retrospective analysis of paroxysmal sympathetic hyperactivity following severe pediatric brain injury.

There are several gaps in the literature related to the prognosis and care of children who have experienced a brain injury then develop paroxysmal sympathetic hyperactivity (PSH).

The study of exercise tests in paroxysmal kinesigenic dyskinesia.

To unravel if there was muscular ion channel dysfunction in paroxysmal kinesigenic dyskinesia (PKD) patients using the exercises tests (ET).

The expanding spectrum of paroxysmal movement disorders: update from clinical features to therapeutics.

This review will discuss the expanding clinical spectrum of paroxysmal movement disorders and therapeutic options in light of emerging genotypic heterogeneity in these conditions.

Incidence of paroxysmal sympathetic hyperactivity following traumatic brain injury using assessment tools.

A consensus statement proposed a diagnostic framework to systematise the identification of paroxysmal sympathetic hyperactivity (PSH) using the PSH-Assessment Measure (PSH-AM).

Tumarkin-like phenomenon as a sign of therapeutic success in benign paroxysmal positional vertigo.

To describe an unusual patient reaction to maneuvers used in the treatment of posterior canal benign paroxysmal positional vertigo (PC-BPPV) that we termed the "Tumarkin-like phenomenon".

Efficacy of Sole Pulmonary Vein Isolation in Patients with Non-paroxysmal Atrial Fibrillation without Significant Left Atrium Scar.

Catheter ablation strategies for non-paroxysmal atrial fibrillation (NPAF) are in varied forms. The mechanisms that circumferential pulmonary vein isolation (CPVI) alone could achieve success in some of the patients with non-paroxysmal atrial fibrillation (NPAF) are not well studied. This study sought to assess the clinical outcome of only CPVI approach in NPAF patients without significant left atrium (LA) scar.

Hospitalization rate of paroxysmal supraventricular tachycardia in Sweden.

The hospitalization rate of paroxysmal supraventricular tachycardia (PSVT) in a nationwide study is not established. We determined age- and sex-specific hospitalization rates and time trends for hospitalized PSVT in the Swedish population between 1987 and 2010.

Evaluation of Benign Paroxysmal Positional Vertigo in American Football Players.

The aim of this investigation was to evaluate the association between posterior channel benign paroxysmal positional vertigo (BPPV) and trauma that is frequently experienced by American football players.

Changes in f-wave characteristics during cryoballoon catheter ablation.

Changes in ECG-derived parameters are studied in atrial fibrillation (AF) patients undergoing cryoballoon catheter ablation. Approach: Parameters characterizing f-wave frequency, morphology by phase dispersion, and amplitude are estimated using a model-based statistical approach. These parameters are studied before, during, and after ablation, as well as for AF type (paroxysmal/persistent). Seventy-seven (49/28 paroxysmal/persistent) AF patients undergoing de novo catheter ablation are included in the...

Benign Paroxysmal Positional Vertigo in Children.

To describe the clinical features of benign paroxysmal positional vertigo (BPPV) in children.

Assessment of sleep quality in benign paroxysmal positional vertigo recurrence.

Despite the availability of highly effective treatments, there is a significant recurrence rate of benign paroxysmal positional vertigo (BPPV). This study is aimed to quantitatively measure sleep quality in BPPV patients and correlate it with the recurrence of BPPV.

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