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镰状细胞贫血患儿、青少年和年轻成人的左心室肥厚

Left ventricular hypertrophy in children, adolescents and young adults with sickle cell anemia.

作者信息

Faro Gustavo Baptista de Almeida, Menezes-Neto Osvaldo Alves, Batista Geodete Santos, Silva-Neto Antônio Pereira, Cipolotti Rosana

机构信息

Universidade Federal de Sergipe (UFS), Aracaju, SE, Brazil.

Universidade Federal de Sergipe (UFS), Aracaju, SE, Brazil.

出版信息

Rev Bras Hematol Hemoter. 2015 Sep-Oct;37(5):324-8. doi: 10.1016/j.bjhh.2015.07.001. Epub 2015 Jul 26.

Abstract

OBJECTIVE

The aims of this study were to estimate the frequency of left ventricular hypertrophy and to identify variables associated with this condition in under 25-year-old patients with sickle cell anemia.

METHODS

A cross-sectional study was performed of children, adolescents and young adults with sickle cell anemia submitted to a transthoracic Doppler echocardiography. The mass of the left ventricle was determined by the formula of Devereux et al. with correction for height, and the percentile curves of gender and age were applied. Individuals with rheumatic and congenital heart disease were excluded. The patients were divided into two groups according to the presence or absence of left ventricular hypertrophy and compared according to clinical, echocardiographic and laboratory variables.

RESULTS

A total of 37.6% of the patients had left ventricular hypertrophy in this sample. There was no difference between the groups of patients with and without hypertrophy according to pathological history or clinical characteristics, except possibly for the use of hydroxyurea, more often used in the group without left ventricular hypertrophy. Patients with left ventricular hypertrophy presented larger left atria and lower hemoglobin and hematocrit levels, reticulocyte index and a higher albumin:creatinine ratio in urine.

CONCLUSION

Left ventricular hypertrophy was observed in more than one-third of the young patients with sickle cell anemia with this finding being inversely correlated to the hemoglobin and hematocrit levels, and reticulocyte index and directly associated to a higher albumin/creatinine ratio. It is possible that hydroxyurea had had a protective effect on the development of left ventricular hypertrophy.

摘要

目的

本研究旨在评估25岁以下镰状细胞贫血患者左心室肥厚的发生率,并确定与该病症相关的变量。

方法

对接受经胸多普勒超声心动图检查的镰状细胞贫血儿童、青少年和年轻成年人进行了一项横断面研究。左心室质量通过Devereux等人的公式计算,并根据身高进行校正,同时应用性别和年龄的百分位数曲线。排除患有风湿性和先天性心脏病的个体。根据是否存在左心室肥厚将患者分为两组,并根据临床、超声心动图和实验室变量进行比较。

结果

在该样本中,共有37.6%的患者存在左心室肥厚。根据病史或临床特征,有左心室肥厚和无左心室肥厚的患者组之间没有差异,可能除了羟基脲的使用,在无左心室肥厚的组中使用更频繁。有左心室肥厚的患者左心房更大,血红蛋白和血细胞比容水平更低,网织红细胞指数更低,尿白蛋白:肌酐比值更高。

结论

在超过三分之一的镰状细胞贫血年轻患者中观察到左心室肥厚,这一发现与血红蛋白和血细胞比容水平以及网织红细胞指数呈负相关,与更高的白蛋白/肌酐比值呈正相关。羟基脲可能对左心室肥厚的发展具有保护作用。

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Sickle-cell disease.镰状细胞病。
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Population estimates of sickle cell disease in the U.S.美国镰状细胞病的人口估计数
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