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[儿童镰状细胞性心脏肥大。13例研究]

[Sickle-cell cardiomegaly in children. Study of 13 cases].

作者信息

Girardet J P, Beauvais P, Laurent F, Perreve-Genet A, Salvadori M C, Bonnet-Gajdos M

出版信息

Arch Fr Pediatr. 1983 Aug-Sep;40(7):525-30.

PMID:6227301
Abstract

Thirteen children, aged 18 months to 14 years and presenting with sickle cell anemia and cardiomegaly (Cardiothoracic ratio greater than or equal to 0.55) were investigated by echocardiography and radio-isotopic measurement of the cardiac output. Eight children presented with one or several criteria of anemic cardiopathy: left ventricular dilatation and hyperkinesia, increased cardiac index. On the contrary, 2 children presented with left ventricular dilatation and decreased contractility, without increase in the cardiac index; 3 children presented with echocardiographic findings similar to those of controls and a normal cardiac index, which is unusual in cases with chronic anemia. These results show that the cardiovascular system of children with sickle cell anemia does not always behave as one would expect in chronic anemia. They also suggest the possible early occurrence of cardiomyopathy, as already described in adults with sickle cell anemia.

摘要

对13名年龄在18个月至14岁之间、患有镰状细胞贫血且有心脏扩大(心胸比率大于或等于0.55)的儿童进行了超声心动图检查和放射性同位素心输出量测量。8名儿童出现了贫血性心脏病的一项或多项标准:左心室扩张和运动亢进,心脏指数增加。相反,2名儿童出现左心室扩张且收缩力下降,心脏指数未增加;3名儿童的超声心动图检查结果与对照组相似且心脏指数正常,这在慢性贫血病例中并不常见。这些结果表明,镰状细胞贫血儿童的心血管系统并不总是表现出人们在慢性贫血中所预期的那样。它们还提示可能早期就出现心肌病,正如在患有镰状细胞贫血的成人中已经描述的那样。

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