地中海贫血儿童生长模式及血清铁蛋白、血红蛋白水平对身体生长影响的前瞻性研究

Bushra Nasreen
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引用次数: 0

摘要

背景:地中海贫血是世界上最常见的遗传性疾病之一,与结构正常血红蛋白合成减少有关。地中海贫血是一种常染色体隐性单基因遗传病。考虑到这一点,本研究旨在研究输血依赖型地中海贫血儿童的生长模式,并评估血清铁蛋白、血红蛋白水平对地中海贫血儿童身体生长模式的影响。方法:对在儿科就诊的52例地中海贫血患者(2 ~ 12岁)进行前瞻性观察研究。对所有患者进行了体重和身高等人体测量。性成熟评级是在女孩10岁和男孩11岁时进行的。实验室参数包括输血前血红蛋白(Hb)和血清铁蛋白。体重、身高和体重指数的百分位数使用世卫组织(2007年)参考数据计算。结果:52例输血依赖型地中海贫血患儿中,6岁以上患儿占多数,35例(67.3%)。平均年龄8.3.05岁。大多数儿童为男性,即32名(61.6%)。平均诊断年龄9.2个月,最小3个月确诊,最大20个月确诊。在52个孩子中,大多数是由非近亲夫妇所生。大多数儿童输血前血红蛋白在5- 7gm %范围内。52例患儿中,61.6% (n=32)患儿血清铁蛋白平均值大于2500,38.4% (n=20)患儿血清铁蛋白平均值小于2500。本研究结果显示,在52名符合IAP标准的儿童中,年龄体重小于50百分位的占86.5% (n=45),大于50百分位的占13.5%(n=7)。年龄体重低于50百分位的45例(86.5%)中,6岁以下儿童占19.2% (n=10), 7 - 12岁儿童占67.3% (n=35)。在年龄体重中
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A prospective study on growth pattern and effect of serum ferritin, haemoglobin levels on physical growth inthalassemic childern
Background: Thalassemia major is one of the most common genetically transmitted diseases in the world and is associated with reduced synthesis of structurally normal hemoglobin. -thalassemia is an autosomal recessive single gene disorder. Keeping this in mind, present study was undertaken to study the growth pattern of transfusion dependent thalassaemic children and to assess the effect serum ferritin, haemoglobin levels on physical growth pattern in thalassemic children. Methods: A prospective observational study was done on 52 thalassaemic patients (aged 2 years-12 years) who attended Department of Pediatrics. Anthropometric measurements like weight and height were taken from all patients. Sexual maturity rating was done in girls 10 years and boys11 years. Lab parameters included pre-transfusion hemoglobin (Hb) and serum ferritin. Percentiles for weight, height and body mass index were calculated using WHO (2007) reference data. Results: Among 52 transfusion dependent thalassemic children majority i.e. 35(67.3%) were more than 6years.Mean age was 8 3.05. Majority of children i.e. 32(61.6%) were male. Mean age of diagnosis was 9.2 months, youngest case being diagnosed at 3rd month, and oldest being at 20 months. Among 52 children most of them are born to non-consanguineous couple. Majority of the children had pre-transfusion haemoglobin in the range of 5-7 gm%. Among 52 children 61.6 % (n=32) had mean serum ferritin more than 2500, and 38.4% (n=20) had mean serum ferritin value less than 2500. The present results showed that, Among 52 children according to IAP standards weight for age less than 50th percentile was 86.5% (n=45) and more than 50th percentile was 13.5%(n=7). Among 45(86.5%) children who had less than 50th percentile weight for age, 19.2 % (n=10) children were less than 6 years of age, remaining 67.3% (n=35) children belongs to 7 to 12 years of age. Among weight for age
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