Hydroxyurea therapy modulates sickle cell anemia red blood cell physiology: Impact on RBC deformability, oxidative stress, nitrite levels and nitric oxide synthase signalling pathway.

Hydroxyurea (HU) has been suggested to act as a nitric oxide (NO) donor in sickle cell anemia (SCA). However, little is known about the HU NO-related effects on red blood cell (RBC) physiology and NO signalling pathway. Thirty-four patients with SCA (22 under HU treatment (HU+) and 12 without (HU-)) and 17 healthy subjects (AA) were included.

Increased Prevalence of Type 2 Diabetes-Related Complications in Combined Type 2 Diabetes and Sickle Cell Trait.

Objective: The prevalence of type 2 diabetes (T2D) is rapidly increasing in sub-Saharan Africa, where sickle cell trait (SCT) is also frequent. Although SCT is generally considered a benign condition, evidence suggests that SCT could exaggerate vascular dysfunction in T2D. However, it remains unclear whether SCT could increase the risk of the development of T2D complications.

Context-dependent dispersal, public information, and heterospecific attraction in newts.

Dispersal is one of the main processes that determine community structure. Individuals make dispersal decisions according to environmental and/or social cues that reflect the fitness prospects in a given patch. The presence and abundance of heterospecifics within the same ecological guild, and/or their breeding success, may act as public information that influences movement decisions.

Large International Validation of ABSIS and PDAI Pemphigus Severity Scores.

The Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity-Score (ABSIS) scores have been proposed to provide an objective measure of pemphigus activity. These scores have been evaluated only on already treated patients mainly with mild to moderate activity. The objective was to assess the interrater reliability of ABSIS and PDAI scores and their correlation with other severity markers in a large international study.

Multiple density-dependent processes shape the dynamics of a spatially structured amphibian population.

Understanding the mechanisms that regulate the dynamics of spatially structured populations (SSP) is a critical challenge for ecologists and conservation managers. Internal population processes such as births and deaths occur at a local level, while external processes such as dispersal take place at an inter-population level. At both levels, density dependence is expected to play a critical role.

Resilience to climate variation in a spatially structured amphibian population.

Understanding the impact of weather fluctuations on demographic parameters is of crucial interest to biodiversity research in a context of global climate change. Amphibians are valuable candidates for investigating this topic due to their strong physiological dependence on water availability and temperature. In this study, we took advantage of data from a long-term capture-mark-recapture (CMR) monitoring program of a great crested newt (Triturus cristatus) population inhabiting a 12-pond archipelago in southeastern France.

Description of 22 new alpha-1 antitrypsin genetic variants.

Alpha-1 antitrypsin deficiency is an autosomal co-dominant disorder caused by mutations of the highly polymorphic SERPINA1 gene. This genetic disorder still remains largely under-recognized and can be associated with lung and/or liver injury. The laboratory testing for this deficiency typically comprises serum alpha-1 antitrypsin quantification, phenotyping according to the isoelectric focusing pattern and genotyping if necessary.

Projections of prevalence, lifetime risk, and life expectancy of Parkinson's disease (2010-2030) in France.

Background: Previous studies on the number of Parkinson's disease (PD) patients in the future based on projections of population size underestimated PD burden because they did not take into account the improvement of life expectancy over time.

Objective: The objective of this study was to assess PD progression from 2010 to 2030 in France in terms of prevalent patient numbers, prevalence rates, lifetime risk, and life expectancy with PD, accounting for projections of overall mortality and increased risk of death of PD patients.

Methods: To provide projections of PD burden, we applied a multistate approach considering age and calendar time to incidence and prevalence rates of PD (France 2010) based on drug claims and national demographic data.

Recurring acute urticaria and abdominal pain: Consider a diagnosis of alpha-galactose anaphylaxis.

Background: Food urticaria is common and generally benign, and it may be of viral or idiopathic aetiology. A food origin of the allergy is frequently sought but rarely found. Mammalian meat anaphylaxis, or alpha-galactose (α-gal) anaphylaxis, is a rare and recently discovered entity.

[Pemphigus and herpes: Multicentre survey and literature review].

Background: Although herpes superinfection is a well-known complication of pemphigus, it has not been widely investigated.

Aim: To investigate the frequency and features of herpes infection in patients with ongoing pemphigus.

Patients And Methods: We carried out a multicenter retrospective study between 2008 and 2016 in patients with newly diagnosed pemphigus presenting active herpes infection.

Determinants of cancer treatment and mortality in older cancer patients using a multi-state model: Results from a population-based study (the INCAPAC study).

Introduction: Several studies have reported disparities in the care management and survival of older cancer patients. The aim of our study was to identify determinants of treatment administration in this population of cancer patients aged over 65 years taking into account competing risks of death.

Methods: The INCAPAC study is a population-based study.

Blood rheology in children with the S/β+-thalassemia syndrome.

The aim of the present study was to compare blood rheological parameters between children with homozygous sickle cell disease (SS), sickle cell SC disease or S/β+-thalassemia syndrome, and healthy children (AA) and to test the associations between blood rheology and the clinical severity in S/β+-thalassemia. Sixty-two SS, 14 SC, 11 S/β+-thalassemia and 12 healthy children participated in this study. Blood viscosity was measured with a cone-plate viscometer at 225 s-1.

Blood rheological abnormalities in sickle cell anemia.

This review focuses on the contribution of abnormal blood rheology in the pathophysiology of sickle cell anemia (SCA). SCA is characterized by a reduction of red blood cell (RBC) deformability but this reduction is very heterogeneous among patients. Recent works have shown that patients with the lowest RBC deformability (measured by ektacytometry) have enhanced hemolysis and would be more prone to develop several complications such as priapism, leg ulcers and glomerulopathy.

Cross sectional study on prevalence of sickle cell alleles S and C among patients with mild malaria in Ivory Coast.

Objectives: Sickle cell anemia is due to a mutations on the betaglobin gene, inducing abnormal hemoglobin. In West Africa the main mutations lead to S or C types of hemoglobin. Patients with homozygote mutations seem protected against severe malaria, but not against mild disease.

A Single-Arm Phase II Trial of Lenalidomide in Relapsing or Refractory Primary Cutaneous Large B-Cell Lymphoma, Leg Type.

Although the combination of rituximab and polychemotherapy has improved prognosis of primary cutaneous diffuse large B-cell lymphoma, leg type, the advanced age of patients limits therapeutic options in relapsing/refractory cases. A multicenter, single-arm, phase II trial was conducted to assess the benefits and safety of lenalidomide in refractory/relapsing primary cutaneous diffuse large B-cell lymphoma, leg type. The primary endpoint was the 6-month overall response rate.

Sex- and age-adjusted prevalence estimates of five chronic inflammatory skin diseases in France: results of the « OBJECTIFS PEAU » study.

Background: There are few population-based studies assessing the prevalence of skin diseases.

Objectives: To estimate the prevalence of five chronic skin inflammatory diseases, i.e.

Estimating dispersal in spatiotemporally variable environments using multievent capture-recapture modeling.

Dispersal is a key process in ecological and evolutionary dynamics. Spatiotemporal variation in habitat availability and characteristics has been suggested to be one of the main cause involved in dispersal evolution and has a strong influence on metapopulation dynamics. In recent decades, the study of dispersal has led to the development of capture-recapture (CR) models that allow movement between sites to be quantified, while handling imperfect detection.

Association between Oxidative Stress, Genetic Factors, and Clinical Severity in Children with Sickle Cell Anemia.

Objectives: To investigate the associations between several sickle cell disease genetic modifiers (beta-globin haplotypes, alpha-thalassemia, and glucose-6-phosphate dehydrogenase deficiency) and the level of oxidative stress and to evaluate the association between oxidative stress and the rates of vaso-occlusive events.

Study Design: Steady-state oxidative and nitrosative stress markers, biological variables, genetic modulators, and vaso-occlusive crisis events requiring emergency admissions were measured during a 2-year period in 62 children with sickle cell anemia (58 SS and 4 Sβ). Twelve ethnic-matched children without sickle cell anemia also participated as healthy controls (AA) for oxidative and nitrosative stress level measurement.

Diagnosis and management of pemphigus: Recommendations of an international panel of experts.

Background: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management.

Objective: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations.

Methods: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus.

Eryptosis and hemorheological responses to maximal exercise in athletes: Comparison between running and cycling.

We compared the effects of cycling and running exercise on hemorheological and hematological properties, as well as eryptosis markers. Seven endurance-trained subjects randomly performed a progressive and maximal exercise test on a cycle ergometer and a treadmill. Blood was sampled at rest and at the end of the exercise to analyze hematological and blood rheological parameters including hematocrit (Hct), red blood cell (RBC) deformability, aggregation, and blood viscosity.

Exploiting rhizosphere microbial cooperation for developing sustainable agriculture strategies.

The rhizosphere hosts a considerable microbial community. Among that community, bacteria called plant growth-promoting rhizobacteria (PGPR) can promote plant growth and defense against diseases using diverse distinct plant-beneficial functions. Crop inoculation with PGPR could allow to reduce the use of pesticides and fertilizers in agrosystems.

Higher Daily Physical Activity Level Is Associated with Lower RBC Aggregation in Carotid Artery Disease Patients at High Risk of Stroke.

Carotid artery disease (CAD) is an atherosclerotic inflammatory disease that affects the arterial wall, specifically at points of bifurcation where blood flow is disturbed. Abnormal blood rheology could participate in the pathophysiology of ischemic cardiovascular disease. Physical activity (PA) is known to improve blood rheology in several chronic disorders.