BackgroundSickle cell anemia (SCA) patients exhibit sub-phenotypes associated to hemolysis and vaso-occlusion. The disease has a chronic inflammatory nature that has been also associated to alterations in the lipid profile. This study aims to analyze hematological and biochemical parameters to provide knowledge about the SCA sub-phenotypes previously described and suggest a dyslipidemic sub-phenotype.MethodsA cross-sectional study was conducted from 2013 to 2014, and 99 SCA patients in steady state were enrolled. We assessed correlations and associations with hematological and biochemical data and investigated the co-inheritance of -α3.7Kb-thalassemia (-α3.7Kb-thal). Correlation analyses were performed using Spearman and Pearson coefficient. The median of quantitative variables between two groups was compared using t-test and Mann-Whitney. P-values <0.05 were considered statistically significant.ResultsWe found significant association of high lactate dehydrogenase levels with decreased red blood cell count and hematocrit as well as high levels of total and indirect bilirubin. SCA patients with low nitric oxide metabolites had high total cholesterol, high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol and reduced very low-density cholesterol, triglycerides, direct bilirubin level and reticulocyte counts. In SCA patients with high-density lipoprotein cholesterol greater than 40 mg/dL, we observed increased red blood cell count, hemoglobin, hematocrit, and fetal hemoglobin and decreased nitric oxide metabolites levels. The presence of -α3.7Kb-thal was associated with high red blood cell count and low mean corpuscular volume, mean corpuscular hemoglobin, platelet count and total and indirect bilirubin levels.ConclusionsOur results provide additional information about the association between biomarkers and co-inheritance of -α3.7Kb-thal in SCA, and suggest the role of dyslipidemia and nitric oxide metabolites in the characterization of this sub-phenotype.
Coronaviruses are single-stranded RNA viruses that cause respiratory and intestinal infections in humans. The coronaviruses (HCoV-NL63, HCoV-229E, HCoV-OC43 and HKU1), were known to cause mild infections in immunocompromised persons. However, at the beginning of the XXI century, two new coronaviruses with high pathogenicity were described; the severe acute respiratory syndrome coronavirus (SARS-CoV) and the middle east respiratory syndrome coronavirus (MERS-CoV). In the Wuhan city, the capital of China's Hubei province in december 2019 a new coronavirus (2019-nCoV) was identified. 1,2 Provisionally named 2019-nCoV, the International Committee on Taxonomy of Viruses (ICTV) was renamed as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The World Health Organization (WHO) named the pathology as coronavirus disease 2019 (COVID-19). 3 The infection by SARS-CoV-2 occurs for respiratory droplets and aerosols released from coughing and sneezing. The viral particles can fall on surfaces and objects, causing the virus to remain on them for several hours. Transmission can occur through direct contact of the hands on mucous membranes of the mouth, nose and eyes. 4,5 Fever, muscle pain, cough and dyspnoea are symptoms frequently reported in patients infected with SARS-CoV-2. 6 A lot of people have a positive prognosis; however, in the elderly population and individuals with underlying chronic diseases (diabetes, hypertension and cardiovascular diseases) the infection causes more serious manifestations, with a high mortality rate. Although the most of patients affected by COVID-19 have mild symptoms, some may develop acute respiratory distress syndrome (ARDS), requiring admission to the intensive care unit (ICU). 7 ARDS is a state of severe acute hypoxia, caused by increased capillary permeability and consequent damage to alveolar cells. 5 Histopathological examinations of lung samples from patients with SARS-CoV-2, are similar to those seen in SARS-CoV and MERS-CoV infections, demonstrating diffuse alveolar damage, fibrinous exudates, pulmonary edema and interstitial inflammatory infiltrates consisting of lymphocytes. 8 The World Health Organization recommends the use of extracorporeal membrane oxygenation (ECMO) in patients affected by COVID-19 with refractory hypoxemia who do not respond to conventional treatment on mechanical ventilation. 9 The ECMO can serve as a rescue therapy for these patients and the venousvenous type is the choice. However, the SARSCov-2 can causes cardiovascular complications and, in these cases, can be considered the configuration venoarterial. 10,11 In patients with SARS-CoV-2, evaluation of candidates for mechanical circulatory support devices is a decision that needs to be careful, factors such as government and hospital policies should be considered. If the medical team feels that ECMO is appropriate, the patient needs to present a favorable prognosis for the use this assistance. These are criteria for ECMO in patients with COVID-19: patients with severe ARDS; Murray Score of 3À4;...
Sickle cell anemia is a severe monogenic disorder characterized by the homozygous state of a single beta globin gene mutation, with heterogeneous clinic characteristics, associated with pro-inflammatory profile, oxidant state and hypercoagulable state. Vessels occlusion is likely initiated by intimal proliferation and amplified by inflammation, excessive adhesion of cells to activated endothelium and vascular tone dysregulation, normally modulated by NFkB pathway both endothelium cells as leukocytes. Herein, we investigated the gene expression of tissue factor (Factor III), oxide nitric synthase (NOS) and endothelial protein C receptor (EPCR) associating with biomarkers of prognosis like hemolysis markers, pro-inflammatory and anti-inflammatory profile. Forty two steady-state sickle cell disease (SCD) patients (16.5 ± 13.5 years, 20 female) from Northeast Brazil were enrolled in this study and were diagnosed in attendance of the outpatients’ clinic of the Sickle Cell Disease Center of Itabuna (CERDOFI). The control group was compound by 20 healthy Brazilian individuals with hemoglobin AA pattern matched by age, years and ethnic origin. The study was approved by the UESC ethical committee and informed consents were signed by patients or official responsible. Using real time quantitative PCR, we analyzed tissue factor, NOS and EPCR gene expression. We also measured hematological and hemoglobin parameters by electronic cell counter and HPLC respectively, biochemical profile was evaluated by colorimetric methodology and cytokine by flow cytometry. The statistical analysis was performed using the Kolmogorov–Smirnov test to access distribution of quantitative variables. Mean values of quantitative variables between groups were compared using an unpaired t-test for data distributed normally and a Mann–Whitney test for non-normal data. Oxide nitric synthase gene expression was increased in SCD patients 1.58-fold compared with healthy controls and higher tissue factor and EPCR gene expression were detected in patients than healthy controls, 4.82-fold and 5.46-fold respectively. The SCD cohort comprised pediatric and adult patients, and the medical history data was search from patient’s records where 95% of patients presented painful crisis at least once. Tissue factor gene expression was positive correlated with expression of NOS (p=0.005) and EPCR (p=0.0001). The increase tissue factor gene expression was detected in patients with high serum levels of bilirubin (p=0.026). Tissue factor gene expression above 75th percentile was associated high concentration of serum creatine kinase and serum calcium (p<0.005) in SCD patients. Our study reveals that genes associate to hemostasis and vascular integrity are upregulated in SCD patients, probably associated to chronic oxidative stress and pro-inflammatory state. Enhanced tissue factor, NOS and EPCR gene expression may influences in the pathophysiology of SCD. Studies tissue factor, NOS and EPCR should be carried out in order to explore mechanism, clarify participation and contributing to search of therapeutic strategies on prevention of vascular events among SCD patients. Disclosures: No relevant conflicts of interest to declare.
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