Nokeaingtong Kwannapas, Charoenkwan Pimlak, Silvilairat Suchaya, Saekho Suwit, Pongprot Yupada, Dejkhamron Prapai
*Department of Pediatrics, Faculty of Medicine†Department of Radiologic Technology, Faculty of Associated Medical Sciences‡Biomedical Engineering Center, Faculty of Engineering, Chiang Mai University, Chiang Mai, Thailand.
J Pediatr Hematol Oncol. 2016 Aug;38(6):457-62. doi: 10.1097/MPH.0000000000000625.
Short stature is one of the most common endocrinopathies in transfusion-dependent thalassemia (TDT). This study aimed to determine the longitudinal pattern of growth in pediatric patients with TDT and study the relationship between growth and hemoglobin level, serum ferritin level/iron overload parameters, and other clinical factors. The interval height-for-age Z-scores (HAZ) of 50 patients with TDT, of a mean age of 13.3±2.8 years, were analyzed using linear mixed model analysis. Nineteen patients (38%) had short stature with HAZ≤-2.0. The prevalence of short stature increased with age. The estimated mean HAZ decreased by 0.19 SD per year from the age of 5 years until approximately 14 years (95% confidence interval [CI], -0.22 to -0.16, P<0.001). Male sex (estimate, -0.28; 95% CI, -0.43 to -0.14; P<0.001), mean 3-year hemoglobin level ≤8 g/dL (estimate, -0.36; 95% CI, -0.53 to -0.19; P<0.001), mean 3-year ferritin level ≥1800 ng/mL (estimate, -0.44; 95% CI, -0.59 to -0.29; P<0.001), and cardiac T2* ≤20 ms (estimate, -1.05; 95% CI, -1.34 to -0.77; P<0.001) were significantly associated with short stature. In conclusion, short stature in patients with TDT is common and relates significantly with increasing age, male sex, hemoglobin level, and iron overload status.
身材矮小是依赖输血的地中海贫血（TDT）患者中最常见的内分泌疾病之一。本研究旨在确定TDT患儿的纵向生长模式，并研究生长与血红蛋白水平、血清铁蛋白水平/铁过载参数以及其他临床因素之间的关系。使用线性混合模型分析对50例平均年龄为13.3±2.8岁的TDT患者的年龄别身高Z评分（HAZ）进行了分析。19例患者（38%）身材矮小，HAZ≤-2.0。身材矮小的患病率随年龄增长而增加。从5岁到大约14岁，估计平均HAZ每年下降0.19标准差（95%置信区间[CI]，-0.22至-0.16，P<0.001）。男性（估计值，-0.28；95%CI，-0.43至-0.14；P<0.001）、平均3年血红蛋白水平≤8g/dL（估计值，-0.36；95%CI，-0.53至-0.19；P<0.001）、平均3年铁蛋白水平≥1800ng/mL（估计值，-0.44；95%CI，-0.59至-0.29；P<0.001）以及心脏T2*≤20ms（估计值，-1.05；95%CI，-1.34至-0.77；P<0.001）与身材矮小显著相关。总之，TDT患者身材矮小很常见，且与年龄增长、男性、血红蛋白水平和铁过载状态显著相关。
