Aortic Intima Media Thickness is Increased and Closely Related to Elevated Oxidative Stress Increases in Beta Thalassemia Minor. / Aumento da Espessura Médio-intimal Aórtica e sua Relação com Estresse Oxidativo Elevado em Pacientes com Talassemia Menor.

BACKGROUND: Abdominal aortic intima media thickness (A-IMT) may be an early marker of subclinical atherosclerosis and an objective indicator of increased oxidative stress in beta-thalassemia minor patients. OBJECTIVE: To evaluate whether aortic and carotid IMTs change with oxidative stress and to assess the relationship between these parameters in beta-thalassemia minor patients. METHODS: The study included 80 patients diagnosed with beta-thalassemia minor, and 50 healthy individuals with similar age and gender. After routine procedures, blood samples were collected from the study groups for thiol-disulfide hemostasis and ischemia-modified albumin (IMA). C-IMT measurements were performed in four different regions (right and left internal and external carotid artery) by ultrasonography. In addition, A-IMT measurement was performed by abdominal ultrasonography. Statistically significant p value was set as <0.05 for all comparisons. RESULTS: In beta-thalassemia minor patients, native thiol, total thiol and native thiol / total thiol ratio were lower, and the IMA, disulfide / native thiol ratio and disulfide / total thiol ratios were higher than in healthy control group. A-IMT measurement was significantly higher in beta-thalassemia minor group than controls (1.46±0.37 vs 1.23±0.22 and p<0.001). When the parameters associated with A-IMT in univariate analysis were evaluated by multivariate linear regression analysis, A-IMT was positively related, and native thiol and total thiol levels were negatively and closely related to IMA (p<0.01). CONCLUSION: We demonstrated, for the first time, that oxidative stress status increased with increased A-IMT, while C-IMT remained unchanged in beta-thalassemia minor patients.

FUNDAMENTO: A espessura médio-intimal (EMI) da artéria aorta abdominal (EMI-A) pode ser um marcador precoce de aterosclerose subclínica e um indicador objetivo de estresse oxidativo em pacientes com talassemia menor. OBJETIVO: Avaliar se as EMIs da artéria aorta e da artéria carótida (EMI-C) se alteram com estresse oxidativo, e examinar a relação entre esses parâmetros em pacientes com talassemia menor. MÉTODOS: O estudo incluiu 80 pacientes diagnosticados com talassemia menor, e 50 indivíduos sadios com idade e sexo similares. Após procedimentos de rotina, as amostras de sangue foram coletadas dos grupos de estudo para a medida da homeostase tiol/dissulfeto e da albumina modificada pela isquemia (AMI). As medidas da EMI-C foram realizadas a partir de quatro regiões diferentes (artéria carótida externa direita e esquerda e artéria carótida interna direita e esquerda) por ultrassonografia, e a medida da EMI-A foi realizada por ultrassonografia abdominal. Um valor de p<0,05 foi definido como estatisticamente significativo. RESULTADOS: Nos pacientes com talassemia menor, os níveis de tiol nativo e tiol total, e a razão tiol nativo/tiol total foram mais baixos, e os valores de AMI, razão dissulfeto/tiol nativo, e razão dissulfeto/tiol total foram mais altos que no grupo controle. A EMI-A foi significativamente maior no grupo de pacientes com talassemia menor que nos controles (1,46±0,37 vs 1,23±0,22 e p<0,001). Quando os parâmetros associados com EMI-A na análise univariada foram avaliados por regressão linear multivariada, EMI-A apresentou uma relação positiva, e os níveis de tiol nativo e tiol total apresentaram uma forte relação negativa com AMI (p<0,01). CONCLUSÃO: Nós demonstramos, pela primeira vez, um aumento no estresse oxidativo com a elevação da EMI-A, e valores inalterados da EMI-C em pacientes com talassemia menor.

Symphysis morphology and mandibular alveolar bone thickness in patients with ß-thalassemia major and different growth patterns.

OBJECTIVE: The present study aimed to assess the morphology of symphysis and alveolar bone thickness (ABT) surrounding mandibular incisors in thalassemic patients, as compared to unaffected individuals. METHODS: This case-control study was conducted on lateral cephalograms of 60 thalassemic and 60 unaffected patients with Class II malocclusion seeking orthodontic treatment at Dental School, Shiraz University of Medical Sciences. The sample was divided into three subgroups including hyperdivergent, normodivergent, and hypodivergent, according to the Jarabak index. Symphysis dimensions and alveolar bone thickness surrounding mandibular incisors were measured using AutoCad software. Finally, the correlation between alveolar bone thickness and symphysis morphology was assessed. RESULTS: In general, chin dimensions and bone thickness at different levels of mandibular incisor roots (cervical, middle, apical) were smaller in thalassemic adolescents than controls. Concerning the total sample as well as the normodivergent subgroup, significantly lower values were observed in thalassemic patients for symphysis width, total ABT at the cervical, and lingual ABT at the apical root area compared to controls (p < 0.05). The hypodivergent growth pattern was not associated with any statistical differences between the groups (p> 0.05). In both thalassemic and control subjects, symphysis width showed a weak to moderate positive correlation with ABT of lower incisors (p< 0.05), whereas symphysis height showed a moderate positive correlation with cervical ABT in only ß-thalassemia patients (p< 0.05). CONCLUSIONS: Compared to controls, ß-thalassemia patients showed thinner alveolar bone at different levels of lower incisor roots and smaller symphysis dimensions. There were significant correlations between symphysis dimensions and alveolar bone thickness of mandibular incisors in the sample.

Results from transcranial Doppler examination on children and adolescents with sickle cell disease and correlation between the time-averaged maximum mean velocity and hematological characteristics: a cross-sectional analytical study.

CONTEXT AND OBJECTIVE: Transcranial Doppler (TCD) detects stroke risk among children with sickle cell anemia (SCA). Our aim was to evaluate TCD findings in patients with different sickle cell disease (SCD) genotypes and correlate the time-averaged maximum mean (TAMM) velocity with hematological characteristics. DESIGN AND SETTING: Cross-sectional analytical study in the Pediatric Hematology sector, Universidade Federal de São Paulo. METHODS: 85 SCD patients of both sexes, aged 2-18 years, were evaluated, divided into: group I (62 patients with SCA/Sß(0) thalassemia); and group II (23 patients with SC hemoglobinopathy/Sß(+) thalassemia). TCD was performed and reviewed by a single investigator using Doppler ultrasonography with a 2 MHz transducer, in accordance with the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. The hematological parameters evaluated were: hematocrit, hemoglobin, reticulocytes, leukocytes, platelets and fetal hemoglobin. Univariate analysis was performed and Pearson's coefficient was calculated for hematological parameters and TAMM velocities (P < 0.05). RESULTS: TAMM velocities were 137 ± 28 and 103 ± 19 cm/s in groups I and II, respectively, and correlated negatively with hematocrit and hemoglobin in group I. There was one abnormal result (1.6%) and five conditional results (8.1%) in group I. All results were normal in group II. Middle cerebral arteries were the only vessels affected. CONCLUSION: There was a low prevalence of abnormal Doppler results in patients with sickle-cell disease. Time-average maximum mean velocity was significantly different between the genotypes and correlated with hematological characteristics.