Role of paraoxonase 1 activity and PON1 gene polymorphisms in sickle cell disease.

Sickle cell disease (SCD) patients often exhibit a dyslipidemic sub-phenotype. Paraoxonase 1 (PON 1) is a serum glycoprotein associated with the high-density lipoproteins cholesterol (HDL-C), and variability in PON1 activity depends on the PON1 genotypes. We investigated the influence of PON1c.

Association of laboratory markers and cerebral blood flow among sickle cell anemia children.

Background: Stroke is one of the highest complications of sickle-cell anemia (SCA). The Transcranial Doppler (TCD) has been adopted worldwide as a gold standard method for detecting alterations in the blood velocity in cerebral arteries. In this study, we investigated the association between laboratory parameters and increased cerebral blood flow velocity in Brazilian SCA pediatric patients.

Effect of lysed and non-lysed sickle red cells on the activation of NLRP3 inflammasome and LTB4 production by mononuclear cells.

Objective And Design: This study tested the hypothesis that sickle red blood cell (SS-RBC) can induce inflammasome NLRP3 components gene expression in peripheral blood mononuclear cells (PBMCs) as well as interleukin-1β (IL-1β) and leukotriene B4 (LTB4) production. Additionally, we investigated the effect of hydroxyurea (HU) treatment in these inflammatory markers.

Methods: PBMCs from healthy donors (AA-PBMC) were challenged with intact and lysed RBCs from SCA patients (SS-RBC) and from healthy volunteers (AA-RBC).

Associations between TGF-1 Levels and Markers of Hemolysis, Inflammation, and Tissue Remodeling in Pediatric Sickle Cell Patients.

Transforming growth factor beta (TGF-) is a cytokine with important involvement in biological processes related to the pathogenesis of sickle cell disease (SCD), including endothelial and vascular dysfunction, inflammation, and hematopoietic homeostasis. This study is aimed at investigating associations between levels of TGF-1 and classical laboratory biomarkers and inflammatory mediators, as well as the tissue inhibitor of metalloproteases-1 (TIMP-1) and matrix metalloproteinase-9 (MMP-9), in pediatric patients ( = 123) with SCD in steady state: 84 with sickle cell anemia (HbSS) and 39 with hemoglobin SC disease (HbSC). A healthy control (HC) group of 59 individuals was also included.

Priapism in sickle cell disease: Associations between NOS3 and EDN1 genetic polymorphisms and laboratory biomarkers.

Priapism is a urologic emergency characterized by an uncontrolled, persistent and painful erection in the absence of sexual stimulation, which can lead to penile fibrosis and impotence. It is highly frequent in sickle cell disease (SCD) associated with hemolytic episodes. Our aim was to investigate molecules that may participate in the regulation of vascular tone.

Hydroxyurea treatment is associated with reduced degree of oxidative perturbation in children and adolescents with sickle cell anemia.

Sickle cell anemia (SCA) is the most common inherited hemolytic anemia worldwide. Here, we performed an exploratory study to investigate the systemic oxidative stress in children and adolescents with SCA. Additionally, we evaluated the potential impact of hydroxyurea therapy on the status of oxidative stress in a case-control study from Brazil.

An evaluation of ticagrelor for the treatment of sickle cell anemia.

Introduction: Ticagrelor is an antiplatelet agent approved for the treatment of patients with an acute coronary syndrome or a history of myocardial infarction. Considering the evidence demonstrating that ticagrelor-mediated inhibition of platelet activation and aggregation have beneficial effects in the treatment of thrombotic conditions, clinical studies have been conducted to evaluate the use of this drug for the treatment of sickle cell disease (SCD), demonstrating satisfactory tolerability and safety.

Areas Covered: Clinical investigation has characterized the pharmacokinetic and pharmacodynamical profile, as well as the efficacy and safety of ticagrelor to prevent painful vaso-occlusive crisis (painful episodes and acute chest syndrome) in SCD patients.

Effect of N(Epsilon)-(carboxymethyl)lysine on Laboratory Parameters and Its Association with Haplotype in Children with Sickle Cell Anemia.

The present study aimed to investigate the association of N -carboxymethyllysine (CML) with laboratory parameters and haplotypes in pediatric sickle cell anemia (SCA) patients with or without hydroxyurea (HU) therapy. We included 55 children with SCA (SCA), where 27 were on HU treatment (SCA-HU) and 28 without HU treatment (SCA-HU). Laboratory characteristics were determined using electronic methods while CML was measured using competitive ELISA.

Hydroxyurea alters hematological, biochemical and inflammatory biomarkers in Brazilian children with SCA: Investigating associations with βS haplotype and α-thalassemia.

This study investigated the effects of hydroxyurea (HU) on hematological, biochemical and inflammatory parameters in children with sickle cell anemia (SCA) in association with βS haplotype and α-thalassemia. We included 22 children with SCA who were followed for an average of 14.5 months.

Hidden hearing loss in children and adolescents with sickle cell anemia.

Objective: To evaluate the auditory system for hidden hearing loss (HHL) and its association with clinical variables and endothelial dysfunction (ED) in children and adolescents with sickle cell anemia (SCA).

Methods: Participants included 37 patients with stable SCA and 44 healthy controls (HC group) (aged 6-18 years) with hearing thresholds ≤ 20 dB (dB) were evaluated for pure tone audiometry, tympanometry, acoustic reflex, otoacoustic emission, and auditory evoked potentials. Laboratory analysis of the lipid profile, and C-reactive protein levels and endothelial function using ultrasonographic imaging of the brachial artery to assess flow-mediated dilation were performed.

Sensorineural hearing loss in children with sickle cell anemia and its association with endothelial dysfunction.

Objectives: To investigate the prevalence of sensorineural hearing loss (SNHL) in children and adolescents with sickle cell anemia (SCA) and its association with endothelial dysfunction (ED).

Methods: Fifty-two participants with stable SCA and 44 apparently healthy (AA genotype) participants aged 6-18 years were evaluated for pure tone audiometry and endothelial function using ultrasonographic imaging of the brachial artery to assess flow-mediated dilation (FMD). Laboratory analysis of the lipid profile and C-reactive protein levels was performed.

The Allele and Anti-D Alloimmunization Present With High Frequency in Brazilian Sickle Cell Disease Patients.

Background: Antigens DIIIa, DAR and DAU are common in people of African descent and are involved in anti-D alloimmunization. Sickle cell disease (SCD) patients frequently need blood therapy and are vulnerable to alloimmunization.

Methods: The study included SCD patients from the Brazilian state of Bahia, which has the highest incidence of the disease in Brazil; 241 SCD patients and 220 healthy individuals were studied.

Associations between endothelial dysfunction and clinical and laboratory parameters in children and adolescents with sickle cell anemia.

Background: Hematological changes can drive damage of endothelial cells, which potentially lead to an early endothelial dysfunction in patients with sickle cell anemia (SCA). An association may exist between endothelial dysfunction and several clinical manifestations of SCA. The present study aims to evaluate the links between changes in endothelial function and clinical and laboratory parameters in children and adolescents with SCA.

Laboratory and Genetic Biomarkers Associated with Cerebral Blood Flow Velocity in Hemoglobin SC Disease.

Reference values for cerebral blood flow velocity (CBFV) in hemoglobin SC disease (HbSC) have not been established. We aimed to investigate associations between laboratory and genetic biomarkers associated with CBFV in HbSC children. Sixty-eight HbSC children were included; CBFV was analyzed by transcranial Doppler, and the time-averaged maximum mean velocity (TAMMV) was estimated.

Heme-mediated cell activation: the inflammatory puzzle of sickle cell anemia.

Hemolysis triggers the onset of several clinical manifestations of sickle cell anemia (SCA). During hemolysis, heme, which is derived from hemoglobin (Hb), accumulates due to the inability of detoxification systems to scavenge sufficiently. Heme exerts multiple harmful effects, including leukocyte activation and migration, enhanced adhesion molecule expression by endothelial cells and the production of pro-oxidant molecules.

Transcranial Doppler in hemoglobin SC disease.

Background: Stroke is a severe clinical disorder in sickle cell disease (SCD), and few studies have evaluated transcranial Doppler (TCD) flow velocities in hemoglobin SC disease (HbSC). The guidelines for stroke risk are based on evaluations in sickle cell anemia (SCA) or HbS/β thalassemia.

Procedure: In this study, we compare cerebral blood flow in patients with SCD stratified by genotypes.

The association of infection and clinical severity in sickle cell anaemia patients.

Sickle cell anaemia (SCA) patients have a high risk of infection. We retrospectively investigated the prevalence of infection among SCA patients from Bahia, Brazil. A total of 1415 SCA patients were studied between 1995 and 2009: 190 (13.

Levels of high-density lipoprotein cholesterol (HDL-C) among children with steady-state sickle cell disease.

Background: The search for sickle cell disease (SCD) prognosis biomarkers is a challenge. These markers identification can help to establish further therapy, later severe clinical complications and with patients follow-up. We attempted to study a possible involvement of levels of high-density lipoprotein cholesterol (HDL-C) in steady-state children with SCD, once that this lipid marker has been correlated with anti-inflammatory, anti-oxidative, anti-aggregation, anti-coagulant and pro-fibrinolytic activities, important aspects to be considered in sickle cell disease pathogenesis.

Clinical, hematological, and molecular characterization of sickle cell anemia pediatric patients from two different cities in Brazil.

This study focused on clinical, hematological, and molecular aspects of sickle cell anemia pediatric patients from two different cites in Brazil. Seventy-one patients from São Paulo and Salvador, aged 3 to 18 years, were evaluated. Hematological analyses, betaS globin gene haplotypes, and alpha2 3.