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重型地中海贫血患者甲状腺功能评估及其在身体生长中的作用。

Assessment of thyroid functions and its role in body growth in thalassemia major.

作者信息

Jain M, Sinha R S, Chellani H, Anand N K

机构信息

Department of Pediatrics, Safdarjang Hospital, New Delhi.

出版信息

Indian Pediatr. 1995 Feb;32(2):213-9.

PMID:8635784
Abstract

The present study was done to establish the role of thyroid gland in causing growth retardation in regularly transfused thalassemic children. Growth, skeletal maturation and thyroid functions were assessed in 25 patients of thalassemia major in the age range of 5-17 years (mean age 10.3 +/ 3.6 years). Thirteen patients were migrants from Pakistan and 12 were of Indian origin. Twenty-five age and sex matched children who were not anemic served as controls. Thalassemic children received multiple blood transfusions ranging from 36-350 units with a mean of 168.4 +/ 98.9 (+/ 1 SD). The mean pretransfusion hemoglobin was 8.7 +/ 1.6 g/dl. Twenty eight per cent patients were below the 5th percentile for height and another 24% between 5th and 10th percentiles. The height age retardation was more pronounced than bone age retardation. The mean serum total T3 and T4 levels were significantly lower (p < 0.001) and the mean serum TSH levels were significantly higher (p < 0.005) in patients with thalassemia major as compared to the controls. Eight patients had high TSH levels; of these 5 had compensated primary subclinical hypothyroidism (elevated TSH with normal T3 and T4) and 3 had uncompensated primary sub-clinical hypothyroidism (elevated TSH, low T4 and normal T3). Two patients had low T4 with normal T3 and TSH levels. Thyroid dysfunction was not related to age, sex, hemoglobin levels and country of origin but transfused iron load (units/kg, units/year) was significantly higher in patients with hypothyroid function compared to those with euthyroid function (p < 0.005). Height age, weight age and bone age retardations were more pronounced in patients with hypothyroid function; however, the difference was not statistically significant. It is concluded that hypothyroidism is unlikely to be the sole cause of growth retardation; however, it may have a potentiating or permissive role. The strong association of high transfused iron load and decreased thyroid function stresses the need for intensive chelation therapy.

摘要

本研究旨在确定甲状腺在导致定期输血的地中海贫血儿童生长发育迟缓中所起的作用。对25例年龄在5至17岁(平均年龄10.3±3.6岁)的重型地中海贫血患者的生长、骨骼成熟度和甲状腺功能进行了评估。13例患者来自巴基斯坦,12例为印度裔。25名年龄和性别匹配的非贫血儿童作为对照。地中海贫血儿童接受了36至350单位的多次输血,平均为168.4±98.9(±1标准差)。输血前平均血红蛋白为8.7±1.6g/dl。28%的患者身高低于第5百分位数,另有24%在第5至第10百分位数之间。身高年龄迟缓比骨龄迟缓更明显。与对照组相比,重型地中海贫血患者的血清总T3和T4水平显著降低(p<0.001),血清TSH水平显著升高(p<0.005)。8例患者TSH水平升高;其中5例为代偿性原发性亚临床甲状腺功能减退(TSH升高,T3和T4正常),3例为失代偿性原发性亚临床甲状腺功能减退(TSH升高,T4降低,T3正常)。2例患者T4降低,T3和TSH水平正常。甲状腺功能障碍与年龄、性别、血红蛋白水平和原籍国无关,但甲状腺功能减退患者的输血铁负荷(单位/千克,单位/年)显著高于甲状腺功能正常的患者(p<0.005)。甲状腺功能减退患者的身高年龄、体重年龄和骨龄迟缓更明显;然而,差异无统计学意义。结论是甲状腺功能减退不太可能是生长发育迟缓的唯一原因;然而,它可能具有增强或促进作用。输血铁负荷高与甲状腺功能降低之间的强关联强调了强化螯合治疗的必要性。

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