Prevention of conversion to abnormal transcranial Doppler with hydroxyurea in sickle cell anemia: A Phase III international randomized clinical trial.

Children with sickle cell anemia (SCA) and conditional transcranial Doppler (TCD) ultrasound velocities (170-199 cm/sec) may develop stroke. However, with limited available clinical data, the current standard of care for conditional TCD velocities is observation. The efficacy of hydroxyurea in preventing conversion from conditional to abnormal TCD (≥200 cm/sec), which confers a higher stroke risk, has not been studied prospectively in a randomized trial. Sparing Conversion to Abnormal TCD Elevation (SCATE #NCT01531387) was a National Heart, Lung, and Blood Institute-funded Phase III multicenter international clinical trial comparing alternative therapy (hydroxyurea) to standard care (observation) to prevent conversion from conditional to abnormal TCD velocity in children with SCA. SCATE enrolled 38 children from the United States, Jamaica, and Brazil [HbSS (36), HbSß(0) -thalassemia (1), and HbSD (1), median age = 5.4 years (range, 2.7-9.8)]. Because of the slow patient accrual and administrative delays, SCATE was terminated early. In an intention-to-treat analysis, the cumulative incidence of abnormal conversion was 9% (95% CI = 0-35%) in the hydroxyurea arm and 47% (95% CI = 6-81%) in observation arm at 15 months (P = 0.16). In post hoc analysis according to treatment received, significantly fewer children on hydroxyurea converted to abnormal TCD velocities when compared with observation (0% vs. 50%, P = 0.02). After a mean of 10.1 months, a significant change in mean TCD velocity was observed with hydroxyurea treatment (-15.5 vs. +10.2 cm/sec, P = 0.02). No stroke events occurred in either arm. Hydroxyurea reduces TCD velocities in children with SCA and conditional velocities.

Newborn screening program for hemoglobinopathies in Rio de Janeiro, Brazil.

BACKGROUND: Newborn screening for hemoglobinopathy in Brazil has been decentralized until 2001 when the Health Ministry of Brazil established the National Newborn Hemoglobinopathy Screening Program. The State of Rio de Janeiro started a program in collaboration with the State Health Department and the Institute of Hematology in Rio (HEMORIO). The goal of this study was to evaluate the effectiveness of the first 10 years of the Newborn Hemoglobinopathy Screening Program in identifying and managing infants with Sickle cell disease (SCD) in the State of Rio de Janeiro. PROCEDURE: Blood samples from 1,217,833 neonates were analyzed by High Performance Liquid Chromatography. Infants with SCD were enrolled in comprehensive treatment programs. RESULTS: Data showed that 4.87% of the newborns were heterozygous for a hemoglobin variant, 0.08% were homozygous or doubly heterozygous for abnormal hemoglobins and 95.02% had normal hemoglobin. All the 912 newborns with SCD were referred for treatment at HEMORIO, 34 (3.7%) of these died due to acute chest syndrome, sepsis or splenic sequestration. Four more children died of unknown causes. The implementation of the Rio de Janeiro Newborn Screening Program gradually increased the area of the State covered by the program. CONCLUSION: Data collected during the 10 years of the program showed reduction in mortality of patients with SCD in comparison to available historical statistical data before the implementation of the national screening program. This 10-year study showed that early diagnosis and treatment of newborns was associated with improved survival and quality of life of Brazilian children with SCD.

The effect of hydroxcarbamide therapy on survival of children with sickle cell disease.

Although evidence is accumulating that hydroxycarbamide decreases mortality among adults with sickle cell disease (SCD), there are no published data regarding the effect of hydroxycarbamide on mortality among children. The Paediatric Hydroxycarbamide Program was established to treat children with SCD aged 3-18 years if they met disease severity criteria. Mortality data and clinical/laboratorial effects of hydroxycarbamide were retrospectively collected for the first 9 years of the Program. Mortality among those who received hydroxycarbamide was compared to that of untreated children. Among 1760 subjects, 267 received hydroxycarbamide at a median dose of 20·8 mg/kg/d (range 10-32) for a median of 2 years (range 0·1-6·5). Survival among hydroxycarbamide-treated children was significantly greater than that among untreated ones (99·5% vs. 94·5%, P = 0·01), due primarily to fewer deaths from acute chest syndrome and infection. Hydroxycarbamide therapy was significantly associated with increases in haemoglobin concentration, fetal haemoglobin, mean corpuscular volume, and reduction in platelet counts, reticulocytes and neutrophils. Toxicity was minimal and predominantly mild reversible neutropenia. Significantly fewer hospitalizations and emergency room visits, and shorter admissions were observed among hydroxycarbamide-treated subjects, when compared to the 12-month period prior to treatment initiation. Hydroxycarbamide therapy reduces disease severity and is probably associated with decreased mortality among children with SCD.

Restrição ao uso de tabaco e a prevenção do câncer bucal / Restriction of tobacco use and the prevention of oral cancer

A Organização Mundial da Saúde relata que a epidemia mundial de tabagismo é uma das maiores ameaças à saúde pública dos tempos modernos. O objetivo deste estudo foi relacionar a adoção de medidas restritivas ao uso do tabaco e suas implicações na prevenção ao câncer bucal, por meio de revisão crítica. As evidencias científicas apontam que o uso de tabaco é considerado como causa primária de muitos problemas e doenças bucais, dentre às quais se destacam o câncer bucal, doença periodontal, perdas dentárias e defeitos congênitos. No Brasil e no mundo os principais documentos que regulam a disposição, uso e estratégias de combate aotabaco são relacionados à legislação vigente, documentos internacionais e aos estudos epidemiológicos. Conclui-se que a prevenção ao câncer bucal está relacionada à conscientização das populações quanto aos malefícios causados por hábitos deletérios, como o uso de tabaco, à adoção de práticas saudáveis, bem como se evidencia a necessidade de desenvolvimento de ações abrangentes para o controle do câncer, nos diferentes níveis de atuação e a adoção de medidas mais amplas de restrição ao uso de tabaco pelos diversossetores da sociedade.

The World Health Organization reports that the worldwide epidemic of smoking is the major threat to the public health of modern times. The objective of this study was to relate the adoption of restrictive measures to tobacco use and its implications in the prevention of oral cancer, through critical review. The scientific evidence suggests that the use of tobacco is considered as the primary cause of many problems and oraldiseases, among which are the oral cancer, periodontal disease, tooth loss and congenital defects. In Brazil and the world the main documents regulating the provision, use and strategies to combat tobacco use are related to legislation, international documents and the epidemiological studies. It is concluded that prevention of oral cancer is related to awareness of people about the harm caused by deleterious habits such as use of tobacco, the adoption of healthy practices, and as well as highlighting the need for development ofcomprehensive actions to control the cancer, the different levels of performance and the adoption of furthermeasures to restrict the use of tobacco by the different sectors of society.

Consumption of bottled water by children in the city of Bauru, State of São paulo, Brazil -- a brief communication.

OBJECTIVES: To determine the consumption of bottled water by children in the city of Bauru, State of São Paulo, Brazil. METHODS: A stratified sample of the 17 areas established by the city plan was used to identify a total of 1,000 homes for visitation. Information was collected using a questionnaire concerning the type of water consumed and population demographics. RESULTS: Overall, around 30 percent of all residences used bottled water. Among all households where bottled water was consumed, about 26 percent had children residing. For those with children and bottled water, 81 percent reported to use bottled water for the preparation of the children's foods and beverages. CONCLUSIONS: An important percentage of children consume bottled water in the city of Bauru, State of São Paulo, Brazil. Considering previous studies showing that fluoride concentrations vary in bottled water, public health measures should be implemented in order to guarantee adequate levels of fluoride in commercialized water.