Outpatient Unit, Hematology and Hemotherapy Center of Bahia, Brazil.
Ethn Dis. 2011 Spring;21(2):243-7.
Hemoglobinopathies are the most common genetic disorders in the world and include sickle cell anemia (SCA), which is a public health problem in Brazil. Nevertheless, the disease is highly unknown among health professionals, and delayed diagnosis constitutes an important cause of concern for caretakers of SCA patients. The purpose of this study was to compare the clinical and laboratory history of SCA patients whose diagnosis was established during the first year of life to those of other SCA patients who had delayed SCA diagnosis. Demographic, clinical, and laboratory data were all reviewed from 99 steady-state SCA patients who were followed in a public hematology and hemotherapy clinic in Salvador, Brazil. The patients were aged > or = 12 years and attended the outpatient unit at least once from November 2008 to June 2009. The data were analyzed in 2010. For all patients, the mean age (+/- SD) at diagnosis was 12.7(+/- 12.1) years, ranging from 0 to 47 years. Mean age was higher in patients whose SCA diagnosis was established after age 5 (32.9 +/- 11.9 years, P = .005). Increased unconjugated bilirubin, stroke and splenic sequestration were more prevalent in patients who were diagnosed in the first year of life (P = .043, .024 and .026 respectively). The data suggest that stroke, splenic sequestration and unconjugated bilirubin level may be influenced by age at SCA diagnosis.
血红蛋白病是世界上最常见的遗传疾病，包括镰状细胞贫血（SCA），这是巴西的一个公共卫生问题。然而，这种疾病在卫生专业人员中知之甚少，延迟诊断是 SCA 患者护理人员关注的一个重要原因。本研究的目的是比较在生命的第一年确诊为 SCA 的患者与其他 SCA 患者的临床和实验室病史，后者的 SCA 诊断被延迟。从巴西萨尔瓦多的一家公共血液学和血液治疗诊所的 99 例稳定状态 SCA 患者中回顾了所有人口统计学、临床和实验室数据。患者年龄≥12 岁，并且在 2008 年 11 月至 2009 年 6 月期间至少一次参加了门诊。数据于 2010 年进行分析。对于所有患者，诊断时的平均年龄（+/-SD）为 12.7（+/-12.1）岁，范围为 0 至 47 岁。在年龄> 5 岁时确诊的患者中，平均年龄较高（32.9 +/- 11.9 岁，P =.005）。在生命的第一年确诊的患者中，未结合胆红素、中风和脾脏隔离更为常见（P =.043，P =.024 和 P =.026）。这些数据表明，中风、脾脏隔离和未结合胆红素水平可能受 SCA 诊断年龄的影响。
