Search citation statements
Paper Sections
Citation Types
Year Published
Publication Types
Relationship
Authors
Journals
ObjectivesTo analyze the frequency of βS-globin haplotypes and alpha-thalassemia, and their influence on clinical manifestations and the hematological profile of children with sickle cell anemia.MethodThe frequency of βS-globin haplotypes and alpha-thalassemia and any association with clinical and laboratorial manifestations were determined in 117 sickle cell anemia children aged 3–71 months. The confirmation of hemoglobin SS and determination of the haplotypes were achieved by polymerase chain reaction-restriction fragment length polymorphism, and alpha-thalassemia genotyping was by multiplex polymerase chain reaction (single-tube multiplex-polymerase chain reaction).ResultsThe genotype distribution of haplotypes was 43 (36.7%) Central African Republic/Benin, 41 (35.0%) Central African Republic/Central African Republic, 20 (17.0%) Rare/atypical, and 13 (11.1%) Benin/Benin. The frequency of the α3.7 deletion was 1.71% as homozygous (−α3.7/−α3.7) and 11.9% as heterozygous (−α3.7/αα). The only significant association in respect to haplotypes was related to the mean corpuscular volume. The presence of alpha-thalassemia was significantly associated to decreases in mean corpuscular volume, mean corpuscular hemoglobin and reticulocyte count and to an increase in the red blood cell count. There were no significant associations of βS-globin haplotypes and alpha-thalassemia with clinical manifestations.ConclusionsIn the study population, the frequency of alpha-thalassemia was similar to published data in Brazil with the Central African Republic haplotype being the most common, followed by the Benin haplotype. βS-globin haplotypes and interaction between alpha-thalassemia and sickle cell anemia did not influence fetal hemoglobin concentrations or the number of clinical manifestations.
ObjectivesTo analyze the frequency of βS-globin haplotypes and alpha-thalassemia, and their influence on clinical manifestations and the hematological profile of children with sickle cell anemia.MethodThe frequency of βS-globin haplotypes and alpha-thalassemia and any association with clinical and laboratorial manifestations were determined in 117 sickle cell anemia children aged 3–71 months. The confirmation of hemoglobin SS and determination of the haplotypes were achieved by polymerase chain reaction-restriction fragment length polymorphism, and alpha-thalassemia genotyping was by multiplex polymerase chain reaction (single-tube multiplex-polymerase chain reaction).ResultsThe genotype distribution of haplotypes was 43 (36.7%) Central African Republic/Benin, 41 (35.0%) Central African Republic/Central African Republic, 20 (17.0%) Rare/atypical, and 13 (11.1%) Benin/Benin. The frequency of the α3.7 deletion was 1.71% as homozygous (−α3.7/−α3.7) and 11.9% as heterozygous (−α3.7/αα). The only significant association in respect to haplotypes was related to the mean corpuscular volume. The presence of alpha-thalassemia was significantly associated to decreases in mean corpuscular volume, mean corpuscular hemoglobin and reticulocyte count and to an increase in the red blood cell count. There were no significant associations of βS-globin haplotypes and alpha-thalassemia with clinical manifestations.ConclusionsIn the study population, the frequency of alpha-thalassemia was similar to published data in Brazil with the Central African Republic haplotype being the most common, followed by the Benin haplotype. βS-globin haplotypes and interaction between alpha-thalassemia and sickle cell anemia did not influence fetal hemoglobin concentrations or the number of clinical manifestations.
Background: Improvements in the iron status of a population as result of food fortification are expected at the long term. In Brazil, the effectiveness of mandatory flour fortification with iron has been evidenced mostly from surveys on gestational anemia after 1 or 2 years from its implementation, in 2004. Our aim was to assess hemoglobin (Hb) concentrations and the prevalences of anemia and linked erythrocyte morphology patterns among pregnant women in 2006 and 2008. Methods: The study design was retrospective and cross-sectional. The analysis was based on secondary data in 546 medical records from women at the times of their first prenatal attendance in 13 public primary healthcare units of the Butantan Administrative Region from São Paulo (SP), Brazil. Anemia was evaluated from Hb concentration (cut-off <11.0 g/dL) and erythrocyte morphology patterns from mean corpuscular volume (MCV), mean corpuscular Hb concentration (MHC), and red cell distribution widths (RDW). Recorded sociodemographic and obstetric data included maternal age, gestational age, ethnoracial self-classification, and residence type. Student's t tests, analysis of variance, Chi-squared tests, and multiple linear and logistic regressions were employed in the statistical analysis using a significance level of 5 %. Results: The prevalence of anemia was 9.7 % in 2006 and 9.4 % in 2008 (P = 0.922), with no significant difference in mean Hb concentrations (P = 0.159). Normocytosis (normal MVC), normochromia (normal MHC), and anisocytosis (high RDW) were found in most anemia cases, suggesting that the low Hb concentrations resulted from mixed causes. In multiple regression analysis, gestational age at the first prenatal attendance was an independent predictor of low Hb and of having anemia. Moreover, black ethnoracial self-classification was associated with lower Hb.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.