Lobo Clarisse Lopes de Castro, Cançado Rodolfo Delfini, Leite Ana Claudia Celestino Bezerra, Dos Anjos Ana Claudia Mendonça, Pinto Ana Cristina Silva, Matta Andre Palma da Cunha, Silva Célia Maria, Silva Gisele Sampaio, Friedrisch João Ricardo, Braga Josefina Aparecida Pellegrini, Lange Marcos Christiano, Figueiredo Maria Stella, Rugani Marília Álvares, Veloso Orlando, Moura Patrícia Gomes, Cortez Paulo Ivo, Adams Robert, Gualandro Sandra Fátima Menosi, de Castilho Shirley Lopes, Thomé Ursula, Zetola Viviane Flumignan
Instituto Estadual de Hematologia Arthur de Siqueira Cavalcanti (HEMORIO), Rio de Janeiro (RJ), Brazil.
Rev Bras Hematol Hemoter. 2011;33(1):43-8. doi: 10.5581/1516-8484.20110014.
Sickle cell disease is the most common monogenic hereditary disease in Brazil. Although strokes are one of the main causes of morbidity and mortality in these patients, the use of transcranial Doppler to identify children at risk is not universally used.
To develop Brazilian guidelines for the use of transcranial Doppler in sickle cell disease children and adolescents, so that related health policies can be expanded, and thus contribute to reduce morbidity and mortality.
The guidelines were formulated in a consensus meeting of experts in transcranial Doppler and sickle cell disease. The issues discussed were previously formulated and scientific articles in databases (MEDLINE, SciELO and Cochrane) were carefully analyzed. The consensus for each question was obtained by a vote of experts on the specific theme.
Recommendations were made, including indications for the use of transcranial Doppler according to the sickle cell disease genotype and patients age; the necessary conditions to perform the exam and its periodicity depending on exam results; the criteria for the indication of blood transfusions and iron chelation therapy; the indication of hydroxyurea; and the therapeutic approach in cases of conditional transcranial Doppler.
The Brazilian guidelines on the use of transcranial doppler in sickle cell disease patients may reduce the risk of strokes, and thus reduce the morbidity and mortality and improve the quality of life of sickle cell disease patients.
镰状细胞病是巴西最常见的单基因遗传病。尽管中风是这些患者发病和死亡的主要原因之一,但使用经颅多普勒来识别高危儿童的做法并未得到普遍应用。
制定巴西关于镰状细胞病儿童和青少年使用经颅多普勒的指南,以便扩大相关卫生政策,从而有助于降低发病率和死亡率。
该指南在经颅多普勒和镰状细胞病专家的共识会议上制定。所讨论的问题事先已拟定,并且对数据库(MEDLINE、SciELO和Cochrane)中的科学文章进行了仔细分析。每个问题的共识通过专家对特定主题的投票获得。
提出了建议,包括根据镰状细胞病基因型和患者年龄使用经颅多普勒的指征;进行检查的必要条件及其根据检查结果的周期性;输血和铁螯合疗法的指征标准;羟基脲的指征;以及有条件经颅多普勒情况下的治疗方法。
巴西关于镰状细胞病患者使用经颅多普勒的指南可能会降低中风风险,从而降低发病率和死亡率,并改善镰状细胞病患者的生活质量。