Misinformation about COVID-19 among middle-aged and older migrants residing in Brazil and Portugal.

OBJECTIVE: The aim of this study was to assess the prevalence of COVID-19 misinformation among migrants aged 50 or older residing in Brazil and Portugal. METHOD: This was a cross-sectional analytical study conducted among migrants from Portuguese-speaking countries living in Brazil and Portugal, who were 50 years of age or older. The prevalence ratios (PR) were estimated using the Poisson regression model. RESULTS: Out of the 304 participants included in the study, 188 (61.8%) agreed with at least one piece of misinformation. Factors such as having a religious affiliation (aPR: 1.24), higher educational attainment (aPR: 1.17), knowing someone who died from COVID-19 (aPR: 1.78), and having no intention to get vaccinated (aPR: 1.36) were associated with a higher likelihood of agreeing with COVID-19 misinformation. CONCLUSION: The findings suggest that access to misinformation was influenced by social, economic, and religious factors among elderly migrants with low digital literacy, thus contributing to the dissemination of false content within this population.

Agreement with COVID-19 disinformation among Portuguese-speaking older adults: an international study.

OBJECTIVES: to assess agreement with COVID-19 disinformation among Portuguese-speaking individuals aged 50 years or older. METHODS: a descriptive and analytical study involving 1,214 older adults born in Portuguese-speaking countries. Data collection occurred through online information mining to recognize COVID-19 and disinformation content, and the application of a structured questionnaire. RESULTS: agreement with disinformation content was 65.2%. Residing outside Brazil is a protective factor for agreement with disinformation content, and those who believe in the truthfulness of the information sources they receive were 31% more likely to agree with disinformation content. CONCLUSIONS: there is a high prevalence of disinformation among the older population in two Portuguese-speaking countries, which should raise the attention of healthcare professionals and guide coping strategies.

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.192Q > R and PON1c.55L > M polymorphisms on PON1 activity and laboratory parameters and the association between PON1 activity and clinical manifestations in SCD patients. We recruited 350 individuals, including 154 SCD patients and 196 healthy volunteers, which comprised the control group. Laboratory parameters and molecular analyses were investigated from the participants' blood samples. We have found increased PON1 activity in SCD individuals compared to the control group. In addition, carriers of the variant genotype of each polymorphism presented lower PON1 activity. SCD individuals carrying the variant genotype of PON1c.55L > M polymorphism had lower platelet and reticulocyte counts, C-reactive protein, and aspartate aminotransferase levels; in addition to higher creatinine levels. SCD individuals carrying the variant genotype of PON1c.192Q > R polymorphism had lower triglyceride, VLDL-c, and indirect bilirubin levels. Furthermore, we observed an association between PON1 activity history of stroke and splenectomy. The present study confirmed the association between PON1c.192Q > R and PON1c.55L > M polymorphisms and PON1 activity, in addition to demonstrate their effects on markers of dislipidemia, hemolysis and inflammation, in SCD individuals. Moreover, data suggest PON1 activity as a potential biomarker related to stroke and splenectomy.

Positive and negative aspects of psychological stress in clinical education in nursing: A scoping review.

BACKGROUND: Even though clinical practice is crucial for nursing students' personal and professional development, it is recurrently associated with stress. However, stressful situations may not only elicit negative responses, but positive responses as well, and such aspects should be further investigated. AIM: To explore the literature addressing the positive and negative aspects of stress experienced by nursing students during clinical practicum. DESIGN: This scoping review followed the Joanna Briggs Institute (JBI) and PRISMA-ScR guidelines. DATA SOURCES: CINAHL, MEDLINE/PubMed, PsycINFO, Web of Science, IBECS, LILACS, BDENF and two theses and dissertations repositories. REVIEW METHODS: Searches were conducted from June to August 2022. Studies addressing the positive and negative aspects of stress experienced by nursing students during clinical practice were included regardless of the method adopted. RESULTS: This review included 32 studies published between 1999 and 2022 in 25 countries across four continents: Africa, America, Asia, and Europe. Stress can positively influence academic performance, especially in mobilizing a search for learning and professional development and in integrating theoretical knowledge and improving clinical skills and competencies. Negatively, stress can cause biopsychosocial symptoms that compromise a student's academic performance and can cause doubts and uncertainties about the construction of their professional identity. CONCLUSIONS: Although the negative aspects stand out over the positive ones, it is evident that both are present in the clinical practice environment. When considering stress of nursing students, educators should conduct the teaching-learning process in order to reduce the negative impact and promote the positive side of stressful situations.

Ensino remoto emergencial na pós-graduação stricto sensu durante a pandemia da covid-19: estudo de reflexão* / Docencia remota de emergencia en posgrado stricto sensu durante la pandemia de covid-19: estudio de reflexión / Emergency remote teaching in stricto sensu graduate courses during the covid-19 pandemic: a reflection study

Objetivo: refletir sobre o ensino remoto, utilizado na pandemia da covid-19, na pós-graduação stricto sensu no contexto da universidade. Método: estudo de reflexão, apoiado em levantamento bibliográfico que permitiu o reconhecimento do predomínio do enfoque técnico, analisado à luz de autores cujo referencial teórico articula a educação e a universidade às relações sociais. Resultados: a análise do ensino remoto demanda uma investigação acerca da sua relação com mudanças que vêm se processando na universidade no cenário neoliberal, valorizando projetos de educação e sociedade com foco mercadológico, influenciando a produção do conhecimento, a formação do pesquisador e do professor da educação superior. Conclusão: ultrapassar a dimensão técnica para a apropriação da dimensão ético-política presente no ensino remoto e nos seus possíveis desdobramentos pós-pandêmico é fundamental, pois o potencial do ato educativo, em termos do encontro entre professor e aluno e da apropriação do conhecimento, não é efetivamente alcançado no ensino remoto.

Objective: to reflect on remote teaching, used during the covid-19 pandemic, in stricto sensu graduate studies in the university context. Method: this is a reflection study, supported by a bibliographical survey that allowed the recognition of the predominance of the technical focus, analyzed in the light of authors whose theoretical framework articulates education and university with social relations. Results: the analysis of remote teaching demands an investigation into its relationship with changes that have been taking place at universities in the neoliberal scenario, valuing education and society projects with a marketing focus, influencing knowledge production, researcher and professor training. Conclusion: going beyond the technical dimension to appropriating the ethical-political dimension present in remote teaching and its possible post-pandemic developments is fundamental, as the educational act's potential, in terms of the meeting between professor and student and appropriation of knowledge, is not effectively achieved in remote teaching.

Objetivo: reflexionar sobre la enseñanza a distancia, utilizada durante la pandemia de covid-19, en los estudios de posgrado stricto sensu en el contexto universitario. Método: se trata de un estudio de reflexión, sustentado en un levantamiento bibliográfico que permitió reconocer el predominio del enfoque técnico, analizado a la luz de autores cuyo marco teórico articula la educación y la universidad con las relaciones sociales. Resultados: el análisis de la enseñanza a distancia exige una investigación sobre su relación con los cambios que se vienen produciendo en la universidad en el escenario neoliberal, valorar proyectos de educación y sociedad con enfoque de marketing, incidiendo en la producción de conocimiento, la formación de investigadores y docentes de educación superior. Conclusión: ir más allá de la dimensión técnica para apropiarse de la dimensión ético-política presente en la enseñanza a distancia y sus posibles desarrollos pospandemia es fundamental, ya que las potencialidades del acto educativo, en cuanto al encuentro entre docente y alumno y la apropiación del conocimiento, no se logra efectivamente en la enseñanza remota.

Misinformation about COVID-19 among middle-aged and older migrants residing in Brazil and Portugal / Desinformación sobre COVID-19 entre migrantes de mediana edad y mayores residentes en Brasil y Portugal / Desinformação sobre COVID-19 entre migrantes de meia-idade e mais velhos residentes no Brasil e em Portugal

ABSTRACT Objective: The aim of this study was to assess the prevalence of COVID-19 misinformation among migrants aged 50 or older residing in Brazil and Portugal. Method: This was a cross-sectional analytical study conducted among migrants from Portuguese-speaking countries living in Brazil and Portugal, who were 50 years of age or older. The prevalence ratios (PR) were estimated using the Poisson regression model. Results: Out of the 304 participants included in the study, 188 (61.8%) agreed with at least one piece of misinformation. Factors such as having a religious affiliation (aPR: 1.24), higher educational attainment (aPR: 1.17), knowing someone who died from COVID-19 (aPR: 1.78), and having no intention to get vaccinated (aPR: 1.36) were associated with a higher likelihood of agreeing with COVID-19 misinformation. Conclusion: The findings suggest that access to misinformation was influenced by social, economic, and religious factors among elderly migrants with low digital literacy, thus contributing to the dissemination of false content within this population.

RESUMEN Objetivo: Evaluar los contenidos de desinformación relacionados con COVID-19 entre migrantes de 50 años o más que residen en Brasil y Portugal. Método: Estudio transversal y analítico con migrantes de países de habla portuguesa que residen en Brasil y Portugal y tienen 50 años o más. Se utilizó el modelo de regresión de Poisson para estimar las razones de prevalencia (RP). Resultados: De los 304 participantes en este estudio, 188 (61,8%) estuvieron de acuerdo con al menos un contenido de desinformación. Tener una religión (aPR:1,24), mayor nivel educativo (aPR:1,17), conocer a alguien que murió a causa de COVID-19 (aPR:1,78) y no tener la intención de vacunarse (aPR:1,36) influyeron en la concordancia con la desinformación sobre COVID-19. Conclusión: El acceso a la desinformación estuvo relacionado con aspectos sociales, económicos y religiosos de los participantes y contribuyó a que la población migrante de edad avanzada y con bajo nivel educativo digital difundiera contenido falso entre otras personas.

RESUMO Objetivo: Avaliar os conteúdos de desinformação relacionados a COVID-19 entre migrantes com 50 ou mais anos de idade residentes no Brasil e em Portugal. Método: Estudo transversal e analítico, com migrantes de países falantes do português residentes no Brasil e em Portugal que possuíam 50 anos ou mais de idade. O modelo de regressão de Poisson foi utilizado para estimar as razões de prevalência (RP). Resultados: Dos 304 participantes deste estudo, 188 (61,8%) concordaram com ao menos um conteúdo de desinformação. Ter uma religião (aPR:1,24), maior escolaridade (aPR:1,17), conhecer alguém que morreu decorrente da COVID-19 (aPR:1,78) e não pretender se vacinar (aPR:1,36) influenciaram a concordância com a desinformação da COVID-19. Conclusão: O acesso às desinformações esteve relacionado com aspectos sociais, econômicos e religiosos dos envolvidos e contribuíram para que a população idosa migratória de baixo letramento digital, pulverizasse conteúdos falsos entre outras pessoas.

Agreement with COVID-19 disinformation among Portuguese-speaking older adults: an international study / Concordancia con contenido de desinformación relacionado con COVID-19 en personas mayores de habla portuguesa: estudio internacional / Concordância com conteúdos de desinformação relacionados à COVID-19 em idosos falantes do português: estudo internacional

ABSTRACT Objectives: to assess agreement with COVID-19 disinformation among Portuguese-speaking individuals aged 50 years or older. Methods: a descriptive and analytical study involving 1,214 older adults born in Portuguese-speaking countries. Data collection occurred through online information mining to recognize COVID-19 and disinformation content, and the application of a structured questionnaire. Results: agreement with disinformation content was 65.2%. Residing outside Brazil is a protective factor for agreement with disinformation content, and those who believe in the truthfulness of the information sources they receive were 31% more likely to agree with disinformation content. Conclusions: there is a high prevalence of disinformation among the older population in two Portuguese-speaking countries, which should raise the attention of healthcare professionals and guide coping strategies.

RESUMEN Objetivos: evaluar la concordancia con contenido de desinformación relacionado con COVID-19 entre personas de 50 años o más de edad, hablantes de portugués. Métodos: estudio descriptivo y analítico con 1.214 personas mayores nacidas en países de habla portuguesa. La recolección de datos se realizó mediante la minería de información en línea para el reconocimiento de contenido de desinformación y COVID-19, y la aplicación de un cuestionario estructurado. Resultados: la concordancia con contenido de desinformación fue del 65,2%. Residir fuera de Brasil es un factor de protección para la concordancia con contenido de desinformación, y aquellos que creen en la veracidad de las fuentes de información que reciben tuvieron un 31% más de probabilidades de estar de acuerdo con contenido de desinformación. Conclusiones: hay una alta prevalencia de desinformación entre la población mayor en dos países de habla portuguesa, lo que debe llamar la atención de los profesionales de la salud y la enfermería y orientar estrategias de enfrentamiento.

RESUMO Objetivos: avaliar a concordância com conteúdos de desinformação relacionados à COVID-19 entre pessoas com 50 anos ou mais de idade, falantes do português. Métodos: estudo descritivo e analítico com 1.214 idosos nascidos em países de língua portuguesa. A coleta de dados ocorreu por meio da mineração de informações online para reconhecimento de conteúdos de desinformação e COVID-19, e aplicação de questionário estruturado. Resultados: a concordância com conteúdos de desinformação foi de 65,2%. Residir fora do Brasil é um fator de proteção para a concordância com conteúdos de desinformação, e aqueles que acreditam na veracidade das fontes de informação que recebem tiveram 31% mais probabilidade de concordar com conteúdos de desinformação. Conclusões: há alta prevalência de desinformação entre a população idosa em dois países de língua portuguesa, o que deve despertar a atenção dos profissionais da saúde e enfermagem, e orientar estratégias de enfrentamento.

Determinants of COVID-19 Vaccine Hesitancy in Portuguese-Speaking Countries: A Structural Equations Modeling Approach.

COVID-19 vaccine hesitancy (VH) has caused concerns due to the possible fluctuations that may occur directly impacting the control of the pandemic. In this study, we aimed to estimate the prevalence and factors associated with COVID-19 VH in Portuguese-speaking countries. We developed a web survey (N:6,843) using an online, structured, and validated questionnaire. We used Measurement Models, Exploratory Factor Analysis, Exploratory Structural Equation Models, and Confirmatory Factor Analysis for the data analysis. The overall prevalence of COVID-19 VH in Portuguese-speaking countries was 21.1%. showed a statistically significant direct effect for VH: vaccine-related conspiracy beliefs (VB) (ß = 0.886), perceived stress (PS) (ß = 0.313), COVID-19 Misinformation (MIS) (ß = 0.259) and individual responses to COVID-19 (CIR) (ß = -0.122). The effect of MIS and CIR for VH was greater among men and of PS and VB among women; the effect of PS was greater among the youngest and of VB and CIR among the oldest. No discrepant differences were identified in the analyzed education strata. In conclusion, we found that conspiracy beliefs related to the vaccine strongly influence the decision to hesitate (not to take or to delay the vaccine). Specific characteristics related to gender, age group, social and cognitive vulnerabilities, added to the knowledge acquired, poorly substantiated and/or misrepresented about the COVID-19 vaccine, need to be considered in the planning of vaccination campaigns. It is necessary to respond in a timely, fast, and accurate manner to the challenges posed by vaccine hesitancy.

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. Eighty eight individuals with SCD were included, of whom thirty-seven reported a history of priapism. Priapism was found to be associated with alterations in laboratory biomarkers, as well as lower levels of HbF. Patients with sickle cell anemia using hydroxyurea and those who received blood products seemed to be less affected by priapism. Multivariate analysis suggested that low HbF and NOm were independently associated with priapism. The frequency of polymorphisms in genes NOS3 and EDN1 was not statistically significant between the studied groups, and the presence of the variant allele was not associated with alterations in NOm and ET-1 levels in patients with SCD. The presence of the variant allele in the polymorphisms investigated did not reveal any influence on the occurrence priapism. Future studies involving larger samples, as well as investigations including patients in priapism crisis, could contribute to an enhanced understanding of the development of priapism in SCD.

Sickle Cell Anemia: Variants in the CYP2D6, CAT, and SLC14A1 Genes Are Associated With Improved Hydroxyurea Response.

Differences in hydroxyurea response in sickle cell anemia may arise due to a series of factors with genetic factors appearing to be predominant. This study aims to investigate the effects of single nucleotide polymorphisms in genes encoding drug-metabolizing enzymes and solute carriers on hydroxyurea response, in patients with sickle cell anemia. For that purpose, a total number of 90 patients with sickle cell anemia were recruited, 45 were undergoing hydroxyurea treatment, while 45 were not under the treatment. Association analyses were performed between CYP3A4 (rs2740574), CYP2D6 (rs3892097), CAT (rs7943316 and rs1001179), and SLC14A1 (rs2298720) variants and laboratory parameters. According to our findings, patients with hydroxyurea treatment demonstrated higher HbF levels and a significant improvement in hemolytic, hepatic, inflammatory, and lipid parameters in comparison to those without the treatment. We also found significant associations between the CYP2D6 (rs3892097), CAT (rs7943316 and rs1001179), and SLC14A1 (rs2298720) variants and an improvement of the therapeutic effects, specifically the hemolytic, hepatic, inflammatory, lipid, and renal parameters. In conclusion, our results highlight the importance of the investigated variants, and their strong association with hydroxyurea efficacy in patients with sickle cell anemia, which may be considered in the future as genetic markers.

TGFBR3 Polymorphisms (rs1805110 and rs7526590) Are Associated with Laboratory Biomarkers and Clinical Manifestations in Sickle Cell Anemia.

Individuals with sickle cell anemia (SCA) present chronic anemia, hemolysis, an exacerbated inflammatory response, and heterogeneous clinical complications, which may be modulated by the transforming growth factor beta (TGF-ß) pathway. Thus, we aimed to investigate polymorphisms (rs1805110 and rs7526590) of the transforming growth factor beta receptor III gene (TGFBR3) with regard to laboratory biomarkers and clinical manifestations in individuals with SCA. Hematological, biochemical, immunological, and genetic analyses were carried out, as well as serum endothelin-1 measurements. The minor allele (A) of the TGFBR3 rs1805110 polymorphism was associated with increased hemoglobin, hematocrit, reticulocyte counts, total cholesterol, low-density lipoprotein, uric acid, and endothelin levels, as well as decreased platelet distribution width (PDW) and the occurrence of bone alterations. The minor allele (T) of TGFBR3 rs7526590 was associated with increased red cell distribution width, PDW, alkaline phosphatase, aspartate aminotransferase, total and indirect bilirubin, and lactate dehydrogenase levels, as well as lower ferritin levels and the occurrence of leg ulcers. Our data suggest that the minor allele (A) of TGFBR3 rs1805110 is associated with inflammation and bone alterations, while the minor allele (T) of TGFBR3 rs7526590 is related to hemolysis and the occurrence of leg ulcers.

Investigation of Lipid Profile and Clinical Manifestations in SCA Children.

INTRODUCTION: Clinical complications in sickle cell anemia (SCA) are heterogeneous and involve several molecules. It has been suggested that SCA individuals present a dyslipidemic phenotype and that lipid parameters are associated with severe clinical complications, such as pulmonary hypertension. We sought to investigate associations between lipid parameters and clinical manifestations, as well as other laboratory parameters in a population of pediatric SCA patients. METHODS: Our cross-sectional evaluation included 126 SCA patients in steady state and who were not undergoing lipid-lowering therapy. Hematological and biochemical parameters were characterized, and previous clinical manifestations were investigated. RESULTS: Total cholesterol and low-density lipoprotein cholesterol (LDL-C) levels were increased in patients with a previous history of pneumonia, which also positively correlated with HbS levels. Decreased LDL-C levels were also associated with leg ulcers and anemia. Elevated high-density lipoprotein cholesterol (HDL-C) levels were associated with pain crises, increased viscosity, and decreased hemolysis. Several studies have determined that lipids play a role in the vascular impairment seen in SCA, which was corroborated by our findings. CONCLUSIONS: In sum, our results suggest that total cholesterol, HDL-C, and LDL-C levels are associated with hemolysis and anemia markers and, most importantly, with clinical complications related to vasculopathy in SCA.

Sickle cell disease: A distinction of two most frequent genotypes (HbSS and HbSC).

Sickle cell disease (SCD) consists of a group of hemoglobinopathies in which individuals present highly variable clinical manifestations. Sickle cell anemia (SCA) is the most severe form, while SC hemoglobinopathy (HbSC) is thought to be milder. Thus, we investigated the clinical manifestations and laboratory parameters by comparing each SCD genotype. We designed a cross-sectional study including 126 SCA individuals and 55 HbSC individuals in steady-state. Hematological, biochemical and inflammatory characterization was performed as well as investigation of previous history of clinical events. SCA patients exhibited most prominent anemia, hemolysis, leukocytosis and inflammation, whereas HbSC patients had increased lipid determinations. The main cause of hospitalization was pain crises on both genotypes. Vaso-occlusive events and pain crises were associated with hematological, inflammatory and anemia biomarkers on both groups. Cluster analysis reveals hematological, inflammatory, hemolytic, endothelial dysfunction and anemia biomarkers in HbSC disease as well as SCA. The results found herein corroborate with previous studies suggesting that SCA and HbSC, although may be similar from the genetic point of view, exhibit different clinical manifestations and laboratory alterations which are useful to monitor the clinical course of each genotype.

Hydroxyurea alters hematological, biochemical and inflammatory biomarkers in Brazilian children with SCA: Investigating associations with ßS 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 ßS haplotype and α-thalassemia. We included 22 children with SCA who were followed for an average of 14.5 months. Laboratory parameters were assessed by electronic methods, and molecular analysis was investigated by PCR-RFLP and allele-specific PCR. Results showed significant increases in hemoglobin, HbF, hematocrit, MCV, MCH, glucose, HDL-C and albumin levels, as well as significant decreases in MCHC and AST levels, WBC, neutrophils, eosinophils, lymphocytes and reticulocytes, in children during HU therapy. HbF levels were positively correlated with hemoglobin, hematocrit, MCV and total protein, yet negatively correlated with MCHC, RDW, AAT and AST during HU therapy (p<0.05). Children who carried the Central African Republic haplotype, in response to HU therapy, presented significant increases in hemoglobin, hematocrit, triglycerides and uric acid levels, as well as significant decreases in MCHC, AST and direct bilirubin levels, WBC, neutrophils, eosinophils, lymphocytes and reticulocytes. Those with the Benin haplotype presented increases in HbF and albumin levels, and a reduction in platelet counts (p<0.05). Children with α-thalassemia presented decreased ALT during HU use, while those without this deletion presented increases in hemoglobin, hematocrit, MCV, MCH, HDL-C and albumin, as well as decreases in MCHC, neutrophils, lymphocytes, reticulocytes and AST (p<0.05). Hence, regardless of its use in association with ßS haplotypes or α-thalassemia, HU seems to be linked to alterations in hemolytic, inflammatory, hepatic, lipid and glycemic profiles.

Hydroxyurea in the management of sickle cell disease: pharmacogenomics and enzymatic metabolism.

Hydroxyurea (HU) was approved to be used in the treatment of sickle cell disease (SCD) because of its anti-sickling potential. However, there is variability in HU response among SCD patients and this can be due to physiological, socioeconomic, environmental, metabolic and/or genetic factors. The present review focuses on the latter two. Three quantitative trait loci, HBG2, BCL11A and HMIP, have been suggested as important markers for HU response. Other genes (ASS1, KLF10, HAO2, MAP3K5, PDE7B, TOX, NOS1, NOS2A, FLT1, ARG1, ARG2, UGT1A1, OR51B5/6, SIN3A, SALL2, SAR1A, UTB, OCTN1, CYP2C9, AQP9, MPO, CYP2E1, and GSTT1) have also been considered. Studies implicate catalase, urease, horseradish peroxidase and enzymes of CYP450 family in HU metabolism. However, little is known about these enzymes. Therefore, further studies are needed to elucidate the metabolic pathway of HU, which will facilitate pharmacogenomic studies and help in identification of candidate genes for predicting HU response.

Genetic Polymorphisms Associated with Environmental Exposure to Polycyclic Derivatives in African Children.

BACKGROUND: The nonracial leukopenia may be a result of exposure to polycyclic derivatives (benzene-toluene-xylene (BTX)) and may arise from a possible change in the bone marrow microenvironment. The present study sought to evaluate the association of genetic polymorphisms in xenobiotic-metabolizing enzymes with hematological and biochemical profiles. METHODS: We evaluated 89 African descendant children, exposed indirectly to benzene derivatives. Laboratory parameters were investigated by automated methods and genetic polymorphisms by PCR-RFLP and PCR multiplex. RESULTS: Children with leukopenia had significantly decreased white blood cells (WBCs) and platelet counts, which is not consistent with benign leukopenia. In the same group, we have found that carriers of the CYP2E1 variant allele had decreased WBC and lymphocytes. Those with NQO1 variant allele had decreased WBC, neutrophil, eosinophil, monocyte, and lymphocyte counts. Carriers of the MPO variant allele had decreased WBC, neutrophil, eosinophil, basophil, monocyte, lymphocyte, and platelet counts and an elevated free iron level. Children with GSTT and GSTM null exhibited decreased WBC, neutrophil, basophil, and lymphocyte counts. Our multivariate analysis model reveals that females were independently associated with leukopenia. CONCLUSION: Our results suggest that the polymorphisms investigated were associated with hematological changes in the studied population. These alterations could be heightened by exposure to benzene derivatives.

Sickle Cell Anemia Patients in Use of Hydroxyurea: Association between Polymorphisms in Genes Encoding Metabolizing Drug Enzymes and Laboratory Parameters.

This study investigated associations between SNPs in genes encoding metabolizing drug enzymes and laboratory parameters in sickle cell anemia patients under hydroxyurea (SCA-HU+). We evaluated hematologic and biochemical parameters by electronic methods and SNPs by PCR-RFLP and multiplex PCR in 35 SCA-HU+ patients and 67 SCA-HU- patients. The HbS, total cholesterol, lactate dehydrogenase, aspartate aminotransferase, total bilirubin and fractions levels, and leukocyte, eosinophil, monocyte, and erythroblast counts were reduced in SCA-HU+ patients (p < 0.05). Moreover, they presented higher HbF, C-reactive protein, and ferritin levels and elevated MCH and MCV values (p < 0.05). Genotype frequencies of variants GA + AA of MPO -463G>A and c1c2 + c2c2 of CYP2E1 -1293G>C/-1053C>T were higher in SCA-HU+ patients (p < 0.05). Independent associations were found between the variant A allele and lower total cholesterol, between c2 allele and low alpha-1 antitrypsin and between the null GSTT1 variant and high indirect and total bilirubin in SCA-HU+ patients. In SCA-HU- patients, independent associations were found between the variant A allele and high uric acid and between c2 allele and high urea. Our results suggest that SNPs MPO -463G>A, CYP2E1 -1293G>C/-1053C>T, and GSTT1 can be associated with alterations in lipid, inflammatory, renal, hemolytic, and hepatic profiles. However, further studies are needed to elucidate these associations.

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. Hematological, biochemical, immunological, and genetic analyses were performed. TAMMV was negatively correlated with red blood cell count (RBC) count, hemoglobin, hematocrit, and direct bilirubin (DB), yet positively correlated with monocytes and ferritin. We found that children with TAMMV ≥ 128 cm/s had decreased red blood cell distribution width (RDW) and nitric oxide metabolite (NOx) concentration. Children with TAMMV ≥ 143.50 cm/s had decreased hemoglobin and hematocrit, as well as increased ferritin levels. Decreased hemoglobin, hematocrit, RDW, and NOx and increased ferritin were detected in children with TAMMV ≥ 125.75 cm/s. The CAR haplotype was associated with higher TAMMV. In association analyses, RBC, hemoglobin, hematocrit, RDW, monocyte, DB, NOx, and ferritin, as well as the CAR haplotype, were found to be associated with higher TAMMV in HbSC children. Multivariate analysis suggested that high TAMMV was independently associated with hematocrit, RDW, and NOx. Additional studies are warranted to validate the establishment of a cutoff value of 125.75 cm/s associated with elevated TAMMV in HbSC children.

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

INTRODUCTION: 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. Area covered: In this review, we describe the effects of heme on leukocytes and endothelial cells, as well as the features of vascular endothelial cells related to vaso-occlusion in SCA. Expert commentary: Free Hb, heme and iron, potent cytotoxic intravascular molecules released during hemolysis, can exacerbate, modulate and maintain the inflammatory response, a main feature of SCA. Endothelial cells in the vascular environment, as well as leukocytes, can become activated via the molecular signaling effects of heme. Due to the hemolytic nature of SCA, hemolysis represents an interesting therapeutic target for heme-scavenging purposes.