重型β地中海贫血患者生长发育分析

Survey on Growth and Development of Patients with Thalassemia Major

目的分析重型β地中海贫血(thalassemia major,TM)患者生长发育状况及其影响因素。方法对365例TM患者测量其身高、体质量并计算其Z值,同时检测输血前血红蛋白(hemoglobin,Hb)、血清铁蛋白(serum ferritin,SF)、甲状腺功能、促性腺激素及性激素浓度,进行相关分析。结果 365例TM患者中,37.5%发育迟缓,7.4%低体质量。发育迟缓发生率随年龄增加而增加,高量输血及低铁负荷患者发育相对较好。38例患者检测甲状腺功能,其中6例患者存在甲状腺功能减退;27例检测性激素水平,15例患者存在性发育障碍。结论 TM患者常伴有生长发育迟缓,主要与治疗不规范有关。TM患者应监测身高、体质量及激素水平。

Objective To analyze the status of growth and development of patients with thalassemia major （TM）. Methods The height and weight of 365 TM patients were measured and the Z value were calculated. The laboratory ex- amination, including hemoglobin （Hb） before transfusion, serum ferritin （SF）, the function of thyroia, the gonadotro- pins and the sexhormone were performed. Results In 365 TM patients, 37. 5 % had growth retardation and 7. 4 % had low weight. The occurrence of growth retardation was increased with the increase of age. Patients with hypertransfusion and low iron-load had better growth. In 38 patients undergoing the thyroid function test, 6 cases had hypothyrosis. In 27 pa- tients undergoing sex hormone test, 15 cases had sex development disorders. Conelusion The TM patients are commonly complicated with growth and development delay and sexual retardation, which is related with non-standarded therapy. The height, weight and endocrine secretion of TM patients should be monitored.