Hematology-Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran.
Endocrinology and Metabolism Research Center, Endocrinology and Metabolism Clinical Sciences Institute, Tehran University of Medical Sciences, Tehrn, Iran.
Minerva Pediatr (Torino). 2021 Oct;73(5):414-419. doi: 10.23736/S2724-5276.16.04553-9. Epub 2016 Jul 14.
Beta thalassemia major and its treatment by hematopoietic stem cell transplantation can have deleterious effects on bone integrity and a main part of such effects is due to their deleterious effects on endocrine systems. So, we assessed the effects of endocrine changes during HSCT (Hematopoietic Stem Cell Transplantation) on growing bones of pediatric thalassemic patients.
Bone-specific alkaline phosphatase and osteocalcin (bone formation markers), N-terminal telopeptide (NTX, bone resorption marker), calcium (Ca), phosphorus (P), alkaline phosphatase (Alk ph), parathyroid hormone (PTH), vitamin D (vit D), prolactin, luteinizing hormone (LH), follicle-stimulating hormone (FSH), thyroxine (T4), triiodothyronine (T3), thyroid-stimulating hormone (TSH), insulin-like growth factor 1 (IGF-1), testosterone (in males) or estradiol (in females), measured in 20 major thalassemic patients with mean age of 10.8±3.9 years. Parameters at the baseline (before HSCT), and 1 month and 3 months after HSCT.
After stem cell transplantation, changes of mean serum levels of NTX, osteocalcin, prolactin, LH, T4, IGF-1, testosterone (in males), Ca, Alk ph, PTH, and vit D were not significant, but bone specific Alk ph, P, T3, TSH, FSH and estradiol changed significantly (P=0.013, P=0.001, P=0.48, P=0.02, P=0.04 and P=0.001, respectively). After one month, there was a significant positive relationship between osteocalcine and T3 (p= 0.009). After 3 months, also, there was a significant positive relationship between osteocalcine and T3 and T4 as well as a negative one with IGF-1 (P<0.001, P<0.02 and P<0.03, respectively).
Endocrine disorders do not appear to have an overall positive or negative effect on bone metabolism (anabolism or catabolism) in HSCT pediatric thalassemic patients in short term (three months).
重型β地中海贫血及其通过造血干细胞移植进行的治疗可能对骨骼完整性产生有害影响，其中一个主要影响因素是其对内分泌系统的有害影响。因此，我们评估了 HSCT（造血干细胞移植）过程中内分泌变化对儿科地中海贫血患者生长中骨骼的影响。
测定 20 例平均年龄为 10.8±3.9 岁的重型地中海贫血患者的骨特异性碱性磷酸酶和骨钙素（骨形成标志物）、N 端肽（骨吸收标志物）、钙（Ca）、磷（P）、碱性磷酸酶（Alk ph）、甲状旁腺激素（PTH）、维生素 D（vit D）、催乳素、促黄体生成素（LH）、促卵泡激素（FSH）、甲状腺素（T4）、三碘甲状腺原氨酸（T3）、促甲状腺激素（TSH）、胰岛素样生长因子 1（IGF-1）、睾酮（男性）或雌二醇（女性），于 HSCT 前（基线）、HSCT 后 1 个月和 3 个月进行测定。
干细胞移植后，NTX、骨钙素、催乳素、LH、T4、IGF-1、睾酮（男性）、Ca、Alk ph、PTH 和 vit D 的血清水平均值变化无统计学意义，但骨特异性 Alk ph、P、T3、TSH、FSH 和雌二醇变化有统计学意义（P=0.013、P=0.001、P=0.48、P=0.02、P=0.04 和 P=0.001）。移植后 1 个月，骨钙素与 T3 之间存在显著正相关（p=0.009）。移植后 3 个月，骨钙素与 T3 和 T4 之间也存在显著正相关，与 IGF-1 之间呈显著负相关（P<0.001、P<0.02 和 P<0.03）。
在短期（3 个月）内，内分泌紊乱似乎对 HSCT 儿科地中海贫血患者的骨代谢（合成代谢或分解代谢）没有总体的积极或消极影响。
