Soliman Ashraf T, El-Matary Wael, Fattah Mohamed M Abdel, Nasr Ibrahim S, El Alaily Rania K, Thabet M Alaa
The Department of Pediatrics, University of Alexandria, Egypt.
Clin Nutr. 2004 Oct;23(5):1153-8. doi: 10.1016/j.clnu.2004.03.001.
Impaired growth accompanying thalassaemia major poses diagnostic and therapeutic problems.
To test the hypothesis that impaired growth of children with thalassaemia major might be corrected, partially or totally, by increasing their caloric intake.
In a prospective controlled study, thirty selected children with thalassaemia major and 30 normal age and sex-matched controls were recruited. The dietary intake of both groups was evaluated. Nutritional status was assessed by measuring the weight, body mass index (BMI), mid-arm circumference (MAC), triceps skin fold thickness (SFT) and serum albumin and insulin-like growth factor-I (IGF-I) concentrations. The thalassaemic group was then, randomly divided into two equal groups. One group was given 8 weeks of high-caloric diet (130-150% of the caloric recommendation for age and sex) and the other was given the normal caloric requirement.
Initially the BMI, triceps SFT and MAC of children with thalassaemia were significantly decreased compared to those for the normal control group. IGF-I and albumin concentrations of thalassemic children before nutritional supplementation (69 +/- 20.5 ng/m and 3.65 +/- 0.67 g/dl, respectively) were significantly lower than those for normal age and sex-matched children (162.5 +/- 24 ng/ml and 4.29 +/- 0.66 g/dl, respectively). After nutritional supplementation for 8 weeks the MAC, SFT and BMI, IGF-I (88.4 +/- 27.3 ng/ml) and albumin concentrations (3.85 +/- 0.85 g/dl) increased significantly (P < 0.05) in the thalassaemic children given the supplementation compared to those without supplementation, however, they were still lower than normal children.
Increased caloric dietary intake increased significantly IGF-I levels in thalassaemic children. This was accompanied with increased BMI, mid-arm circumference and skin fold thickness. Growth impairment of children with thalassaemia major, without endocrinopathy and/or cardiomyopathy, can be partially corrected by increasing caloric intake.
重型地中海贫血伴发的生长发育障碍带来了诊断和治疗方面的问题。
检验如下假设，即通过增加热量摄入，重型地中海贫血患儿生长发育障碍可能会得到部分或完全纠正。
在一项前瞻性对照研究中，选取了30名重型地中海贫血患儿以及30名年龄、性别匹配的正常对照儿童。评估了两组的饮食摄入量。通过测量体重、体重指数（BMI）、上臂中部周长（MAC）、三头肌皮褶厚度（SFT）以及血清白蛋白和胰岛素样生长因子-I（IGF-I）浓度来评估营养状况。然后将地中海贫血组随机分为两组。一组给予8周高热量饮食（为年龄和性别推荐热量的130 - 150%），另一组给予正常热量需求饮食。
最初，与正常对照组相比，地中海贫血患儿的BMI、三头肌SFT和MAC显著降低。营养补充前，地中海贫血患儿的IGF-I和白蛋白浓度（分别为69±20.5 ng/ml和3.65±0.67 g/dl）显著低于年龄、性别匹配的正常儿童（分别为162.5±24 ng/ml和4.29±0.66 g/dl）。营养补充8周后，给予补充的地中海贫血患儿的MAC、SFT和BMI、IGF-I（88.4±27.3 ng/ml）和白蛋白浓度（3.85±0.85 g/dl）与未补充的患儿相比显著增加（P < 0.05），然而，它们仍低于正常儿童。
增加热量饮食摄入可显著提高地中海贫血患儿的IGF-I水平。这伴随着BMI、上臂中部周长和皮褶厚度的增加。对于无内分泌病和/或心肌病的重型地中海贫血患儿，生长发育障碍可通过增加热量摄入得到部分纠正。
