Sickle cell anemia - Nitric oxide related genetic modifiers of hematological and biochemical parameters.

BACKGROUND: Sickle cell anemia (SCA) is an inherited blood disorder. SCA patients present clinical and hematologic variability that cannot be only explained by the single mutation in the beta-globin gene. Others genetic modifiers and environmental effects are important for the clinical phenotype. SCA patients present arginine deficiency that contributes to a lower nitric oxide (NO) bioactivity. OBJECTIVE: The aim of this work is to determine the association between hematological and biochemical parameters and genetic variants from eNOS gene, in pediatric SCA patients. METHODS: 26 pediatric SCA patients were genotyped using polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) techniques in three important eNOS gene polymorphisms - rs2070744, rs1799983 and intron 4 VNTR. RESULTS: Results from this study show a significant statistical association between some parameters and genetic variants: an increased reticulocyte count and high serum lactate dehydrogenase levels were associated with both the rs2070744_TT and the rs1799983_GG genotypes at eNOS gene and high levels of neutrophils were associated with the eNOS4a allele at intron 4 VNTR. CONCLUSIONS: Our results reinforce the importance of NO bioactivity in SCA. We presume that NO, and its precursors might be used as therapy to improve the quality of life of SCA patients.

The impact of clinical use on the torsional behavior of Reciproc and WaveOne instruments.

OBJECTIVE: The aim of this study was to assess the influence of clinical use, in vivo, on the torsional behavior of Reciproc and WaveOne instruments considering the possibility that they degraded with use. MATERIAL AND METHODS: Diameter at each millimeter, pitch length, and area at 3 mm from the tip were determined for both types of instruments. Twenty-four instruments, size 25, 0.08 taper, of each system were divided into two groups (n=12 each): Control Group (CG), in which new Reciproc (RC) and WaveOne Primary (WO) instruments were tested in torsion until rupture based on ISO 3630-1; and Experimental Group (EG), in which each new instrument was clinically used to clean and shape the root canals of one molar. After clinical use, the instruments were analyzed using optical and scanning electron microscopy and subsequently tested in torsion until fracture. Data were analyzed using one-way analysis of variance at a=.05. RESULTS: WO instruments showed significantly higher mean values of cross-sectional area A3 (P=0.000) and smaller pitch lengths than RC instruments with no statistically significant differences in the diameter at D3 (P=0.521). No significant differences in torsional resistance between the RC and WO new instruments (P=0.134) were found. The clinical use resulted in a tendency of reduction in the maximum torque of the analyzed instruments but no statistically significant difference was observed between them (P=0.327). During the preparation of the root canals, two fractured RC instruments and longitudinal and transversal cracks in RC and WO instruments were observed through SEM analysis. CONCLUSION: After clinical use, no statistically significant reduction in the torsional resistance was observed.

The impact of clinical use on the torsional behavior of Reciproc and WaveOne instruments

ABSTRACT Torsional overload is a fracture representative parameter for instruments in single-file techniques. Objective The aim of this study was to assess the influence of clinical use, in vivo, on the torsional behavior of Reciproc and WaveOne instruments considering the possibility that they degraded with use. Material and Methods Diameter at each millimeter, pitch length, and area at 3 mm from the tip were determined for both types of instruments. Twenty-four instruments, size 25, 0.08 taper, of each system were divided into two groups (n=12 each): Control Group (CG), in which new Reciproc (RC) and WaveOne Primary (WO) instruments were tested in torsion until rupture based on ISO 3630-1; and Experimental Group (EG), in which each new instrument was clinically used to clean and shape the root canals of one molar. After clinical use, the instruments were analyzed using optical and scanning electron microscopy and subsequently tested in torsion until fracture. Data were analyzed using one-way analysis of variance at a=.05. Results WO instruments showed significantly higher mean values of cross-sectional area A3 (P=0.000) and smaller pitch lengths than RC instruments with no statistically significant differences in the diameter at D3 (P=0.521). No significant differences in torsional resistance between the RC and WO new instruments (P=0.134) were found. The clinical use resulted in a tendency of reduction in the maximum torque of the analyzed instruments but no statistically significant difference was observed between them (P=0.327). During the preparation of the root canals, two fractured RC instruments and longitudinal and transversal cracks in RC and WO instruments were observed through SEM analysis. Conclusion After clinical use, no statistically significant reduction in the torsional resistance was observed.

Impact of heat treatments on the fatigue resistance of different rotary nickel-titanium instruments.

INTRODUCTION: The aim of this study was to assess the influence of M-Wire (Dentsply Tulsa Dental Specialties, Tulsa, OK) and controlled memory technologies on the fatigue resistance of rotary nickel-titanium (NiTi) files by comparing files made using these 2 technologies with conventional NiTi files. METHODS: Files with a similar cross-sectional design and diameter were chosen for the study: new 30/.06 files of the EndoWave (EW; J. Morita Corp, Osaka, Japan), HyFlex (HF; Coltene/Whaledent, Inc, Cuyahoga Falls, OH), ProFile Vortex (PV; Dentsply Tulsa Dental Specialties, Tulsa, OK), and Typhoon (TYP; Clinician's Choice Dental Products, New Milford, CT) systems together with ProTaper Universal F2 instruments (PTU F2; Dentsply Maillefer, Ballaigues, Switzerland). The compositions and transformation temperatures of the instruments were analyzed using x-ray energy-dispersive spectroscopy and differential scanning calorimetry, whereas the mean file diameter values at 3 mm from the tip (D3) were measured using image analysis software. The average number of cycles to failure was determined using a fatigue test device. RESULTS: X-ray energy-dispersive spectroscopy analysis showed that, on average, all the instruments exhibited the same chemical composition, namely, 51% Ni-49% Ti. The PV, TYP, and HF files exhibited increased transformation temperatures. The PTU F2, PV, and TYP files had similar D3 values, which were less than those of the EW and HF files. The average number of cycles to failure values were 150% higher for the TYP files compared with the PV files and 390% higher for the HF files compared with the EW files. CONCLUSIONS: M-Wire and controlled memory technologies increase the fatigue resistance of rotary NiTi files.

Portuguese Children's Sleep Habits Questionnaire -validation and cross-cultural comparison / Questionário de Hábitos de Sono das Crianças em Português - validação e comparação transcultural

OBJECTIVE: To validate the Portuguese version of the Children's Sleep Habits Questionnaire (CSHQ-PT) and compare it to the versions from other countries. METHODS: The questionnaire was previously adapted to the Portuguese language according to international guidelines. 500 questionnaires were delivered to the parents of a Portuguese community sample of children aged 2 to 10 years old. 370 (74%) valid questionnaires were obtained, 55 children met exclusion criteria and 315 entered in the validation study. RESULTS: The CSHQ-PT internal consistency (Cronbach's α) was 0.78 for the total scale and ranged from 0.44 to 0.74 for subscales. The test-retest reliability for subscales (Pearson's cor-relations, n=58) ranged from 0.59 to 0.85. Our data did not adjust to the original 8 domains structure in Confirmatory Factor Analysis but the Exploratory Factor Analysis extracted 5 factors that have correspondence to CSHQ subscales. CONCLUSION: The CSHQ-PT evidenced psychometric properties that are comparable to the versions from other countries and adequate for the screening of sleep disturbances in children from 2 to 10 years old. .

OBJETIVO: Validar a versão em português do Questionário de Hábitos de Sono das Crianças (CSHQ-PT) e compará-la às versões de outros países. MÉTODOS: O questionário foi anteriormente adaptado para o português de acordo com as diretrizes internacionais. 500 questionários foram entregues aos pais de uma amostra populacional portuguesa de crianças com idade entre 2 e 10 anos. 370 (74%) questionários válidos foram obtidos, 55 crianças apresentaram critérios de exclusão e 315 foram aceitas no estudo de validação. RESULTADOS: A consistência interna do CSHQ-PT (α de Cronbach) foi de 0,78 para a escala completa e variou de 0,44 a 0,74 nas subescalas. A confiabilidade teste-reteste das subescalas (correlações de Pearson, n = 58) variou de 0,59 a 0,85. Nossos dados não foram compatíveis com a estrutura original de 8 domínios na Análise Fatorial Confirmatória, porém a Análise Fatorial Exploratória extraiu 5 fatores que correspondem a subescalas do CSHQ. CONCLUSÃO: O CSHQ-PT apresentou propriedades psicométricas comparáveis às versões de outros países e adequadas para triagem de problemas do sono em crianças de 2 a 10 anos de idade. .

Portuguese Children's Sleep Habits Questionnaire - validation and cross-cultural comparison.

OBJECTIVE: To validate the Portuguese version of the Children's Sleep Habits Questionnaire (CSHQ-PT) and compare it to the versions from other countries. METHODS: The questionnaire was previously adapted to the Portuguese language according to international guidelines. 500 questionnaires were delivered to the parents of a Portuguese community sample of children aged 2 to 10 years old. 370 (74%) valid questionnaires were obtained, 55 children met exclusion criteria and 315 entered in the validation study. RESULTS: The CSHQ-PT internal consistency (Cronbach's α) was 0.78 for the total scale and ranged from 0.44 to 0.74 for subscales. The test-retest reliability for subscales (Pearson's correlations, n=58) ranged from 0.59 to 0.85. Our data did not adjust to the original 8 domains structure in Confirmatory Factor Analysis but the Exploratory Factor Analysis extracted 5 factors that have correspondence to CSHQ subscales. CONCLUSION: The CSHQ-PT evidenced psychometric properties that are comparable to the versions from other countries and adequate for the screening of sleep disturbances in children from 2 to 10 years old.

Early modification of sickle cell disease clinical course by UDP-glucuronosyltransferase 1A1 gene promoter polymorphism.

Elevated erythrocyte destruction in sickle cell disease (SCD) results in chronic hyperbilirubinaemia and, in a subset of patients, cholelithiasis occurs. We investigated whether the (TA)n promoter polymorphism in the UDP-glucuronosyltransferase 1A1 gene (UGT1A1) may modify bilirubin metabolism, influencing bilirubinaemia, predisposition to cholelithiasis and subsequent cholecystectomy, in a group of 153 young SCD patients (mean age 12.0 +/- 9.0 years) predominantly of Bantu beta S haplotype. The concomitant effect of alpha thalassaemia was also analysed. Among the several UGT1A1 genotypes found, the most frequent were the (TA)6/(TA)6 (n = 37), (TA)6/(TA)7 (n = 60) and (TA)7/(TA)7 (n = 29). These groups of patients did not significantly differ in age, gender ratio and haemoglobin, foetal haemoglobin and reticulocyte levels. On the other hand, total bilirubin levels were significantly different between groups, with an increased (TA) repeat number being associated with higher bilirubinaemia. Furthermore, both cholelithiasis and cholecystectomy were more frequent in groups with higher (TA) repeat number, although the former association was not statistically significant. None of the mentioned parameters is statistically different within UGT1A1 groups with the presence of alpha thalassaemia. Thus, the UGT1A1 promoter polymorphism may represent an important nonglobin genetic modifier of Bantu SCD patients' clinical manifestations, even at a young age.

Conhecimento de gestantes sobre o exame anti-hiv no pré-natal / Pregnant mothers’ awareness about the anti-hiv test during prenatal care visists

A epidemia da Aids atinge cada vez mais mulheres em idade fértil. A realização do exame anti-HIV no pré-natal é uma importante estratégia para reduzir a morbi-mortalidade por esta causa. O objetivo foi investigar o conhecimento das gestantes quanto à importância da realização do exame anti-HIV no pré-natal. Estudo descritivo com 50 gestantes maiores de 18 anos, que realizavam pré-natal de baixo risco em consulta subseqüente sem diagnóstico firmado para Aids. A idade das gestantes predominou entre 20 a 25 anos; a maioria tinha sido abordada em consultas anteriores a importância sobre o exame anti-HIV e 92% confirmaram ter realizado esse exame após a primeira consulta de pré-natal. Reafirmam-se os benefícios deste exame no pré-natal e a importância da educação em saúde na prevenção e no controle da doença.

Clinical and laboratory effects of hydroxyurea in children and adolescents with sickle cell anemia: a Portuguese hospital study.

Our aim was to assess the efficacy and safety of hydroxyurea (HU) in children with severe forms of sickle cell anemia followed in a Portuguese hospital. We carried out an open-label uncontrolled prospective study, which included children with severe forms of sickle cell anemia. Hydroxyurea was started at 15 mg/kg/day and increased to a maximum dose of 25 mg/kg/day. Patients were monitored to assess compliance, clinical and hematological response and toxicity. Nine children and adolescents, five girls and four boys, with a median age of 13 years (range 8 to 16) were enrolled in the study during a period of 24 months. All patients completed at least 15 months of therapy. Hb F was significantly increased, from a mean of 7.0 +/- 3.9% to 13.7 +/- 5.3% (p = 0.028). Clinically, all patients responded significantly with a reduction of 80% in the number of vaso-occlusive crises (VOC), 69% in hospital admissions, 76% in hospitalization days and 67% in transfusion requirements, without significant toxicity. We concluded that, in our population, HU proved to be effective in increasing Hb F levels, and in decreasing hospitalizations for VOC and transfusion requirements with no major side effects. Long-term clinical follow-up is important to certify benefit maintenance.