埃及镰状细胞贫血患儿的甲状腺功能与铁负荷的相关性
Thyroid Function in Egyptian Children with Sickle Cell Anemia in Correlation with Iron Load.
作者信息
Hagag Adel A, El-Asy Hassan M, Badraia Ibrahim M, Hablas Nahed M, El-Latif Amal Ezzat Abd
机构信息
Pediatrics Departments, Faculty of Medicine, Tanta University, Tanta, Gharbia, Egypt.
Clinical Pathology Departments, Faculty of Medicine, Tanta University, Tanta, Gharbia, Egypt.
出版信息
Endocr Metab Immune Disord Drug Targets. 2019;19(1):46-52. doi: 10.2174/1871530318666180912153349.
BACKGROUND
Sickle Cell Disease (SCD) is characterized by defective hemoglobin synthesis, hemolytic anemia, frequent thrombosis and chronic organ damage including endocrine organs.
AIM
To assess thyroid function in children with SCD in correlation and iron load.
PATIENTS AND METHOD
This study was conducted on 40 children with SCD with iron overload (serum ferritin more than 1000 ng/ml) including 22 males and 18 females with their ages ranging from 11-14 years and mean age value of 11.63±1.36 years and 40 healthy children of matched age and sex as a control group. For all patients; complete blood count, hemoglobin electrophoresis, serum ferritin, serum iron, iron binding capacity and thyroid function including Free Thyroxine (FT4), Free Triiodothyronine (FT3), Thyroid Stimulating Hormone (TSH), Thyroid Peroxidase Antibody (TPOAb) and Thyroglobulin Antibody (TgAb) were done.
RESULTS
Significantly higher serum ferritin and iron and significantly lower Total Iron Binding Capacity (TIBC) were found in patients compared with controls (mean serum ferritin was 1665.2±1387.65ng/ml in patients versus 192.55±107.2ng/ml in controls with p-value of 0. 007, mean serum iron was 164±83.9 ug/dl in patients versus 89.5±4.5ug/dl in controls with p-value of 0.039, mean TIBC was 238±44.5ug/dl in patients versus 308±11ug/dl in controls with p-value of 0.001). Significantly higher serum TSH and significantly lower Free T3 and Free T4 were found in patients compared with controls with no significant correlation between thyroid hormones and serum ferritin (mean serum TSH was 4.61±1.2 µIU/mL in patients versus 2.11 ± 0.54 µIU /mL in controls with p-value of 0. 045, mean serum FT3 was 2.61 ±1.3 pg/mL versus 3.93±0.47pg/mL in controls with p-value of 0.027, mean serum FT4 was 0.91±0.174 ng/dL versus 1.44± 0.164 ng/dLin controls with p-value of 0.047, r = - 0. 008 and p-value was 0. 973 for correlation between free T4 and serum ferritin, r = -0. 028 and p-value was 0. 9 for correlation between TSH and serum ferritin and r= - 0.259 and p-value was 0.27 for correlation betweenT3 and serum ferritin). There were no significant differences between patients and controls regarding thyroid peroxidase antibody and thyroglobulin antibody (mean serum thyroid peroxidase antibody was 22.45± 4.32 in patients versus 22.45 ± 3.21 in controls with p-value of 0.98 while mean serum thyroglobulin antibody was 12.32 ± 2.65 in patients versus 12.99 ± 2.34 in controls with p-value of 0.76.
CONCLUSION
Thyroid hormones deficiency may occur in some patients with SCD.
RECOMMENDATIONS
Regular assessment of thyroid function in children with SCD may be recommended as they are more vulnerable to develop hypothyroidism and may require replacement therapy.
背景
镰状细胞病（SCD）的特征是血红蛋白合成缺陷、溶血性贫血、频繁血栓形成以及包括内分泌器官在内的慢性器官损伤。
目的
评估SCD患儿的甲状腺功能及其与铁负荷的相关性。
患者与方法
本研究对40例铁过载的SCD患儿（血清铁蛋白超过1000 ng/ml）进行了研究，其中包括22名男性和18名女性，年龄在11至14岁之间，平均年龄为11.63±1.36岁，同时选取40名年龄和性别匹配的健康儿童作为对照组。对所有患者进行了全血细胞计数、血红蛋白电泳、血清铁蛋白、血清铁、铁结合能力以及甲状腺功能检查，包括游离甲状腺素（FT4）、游离三碘甲状腺原氨酸（FT3）、促甲状腺激素（TSH）、甲状腺过氧化物酶抗体（TPOAb）和甲状腺球蛋白抗体（TgAb）。
结果
与对照组相比，患者的血清铁蛋白和铁显著升高，总铁结合能力（TIBC）显著降低（患者的平均血清铁蛋白为1665.2±1387.65 ng/ml，对照组为192.55±107.2 ng/ml，p值为0.007；患者的平均血清铁为164±83.9 μg/dl，对照组为89.5±4.5 μg/dl，p值为0.039；患者的平均TIBC为238±44.5 μg/dl，对照组为308±11 μg/dl，p值为0.001）。与对照组相比，患者的血清TSH显著升高，游离T3和游离T4显著降低，甲状腺激素与血清铁蛋白之间无显著相关性（患者的平均血清TSH为4.61±1.2 μIU/mL，对照组为2.11±0.54 μIU/mL，p值为0.045；患者的平均血清FT3为2.61±1.3 pg/mL，对照组为3.93±0.47 pg/mL，p值为0.027；患者的平均血清FT4为0.91±0.174 ng/dL，对照组为1.44±0.本研究对40例铁过载的SCD患儿（血清铁蛋白超过1000 ng/ml）进行了研究，其中包括22名男性和18名女性，年龄在11至14岁之间，平均年龄为11.63±1.36岁，同时选取40名年龄和性别匹配的健康儿童作为对照组。对所有患者进行了全血细胞计数、血红蛋白电泳、血清铁蛋白、血清铁、铁结合能力以及甲状腺功能检查，包括游离甲状腺素（FT4）、游离三碘甲状腺原氨酸（FT3）、促甲状腺激素（TSH）、甲状腺过氧化物酶抗体（TPOAb）和甲状腺球蛋白抗体（TgAb）。
结果
与对照组相比，患者的血清铁蛋白和铁显著升高，总铁结合能力（TIBC）显著降低（患者的平均血清铁蛋白为1665.2±1387.65 ng/ml，对照组为192.55±107.2 ng/ml，p值为0.007；患者的平均血清铁为164±83.9 μg/dl，对照组为89.5±4.5 μg/dl，p值为0.039；患者的平均TIBC为238±44.5 μg/dl，对照组为308±11 μg/dl，p值为0.001）。与对照组相比，患者的血清TSH显著升高，游离T3和游离T4显著降低，甲状腺激素与血清铁蛋白之间无显著相关性（患者的平均血清TSH为4.61±1.2 μIU/mL，对照组为2.11±0.54 μIU/mL，p值为0.045；患者的平均血清FT3为2.61±1.3 pg/mL，对照组为3.93±0.47 pg/mL，p值为0.027；患者的平均血清FT4为0.91±0.174 ng/dL，对照组为1.44±0.164 ng/dL，p值为0.047；游离T4与血清铁蛋白的相关性r = -0.008，p值为0.973；TSH与血清铁蛋白的相关性r = -0.028，p值为0.9；T3与血清铁蛋白的相关性r = -0.259，p值为0.27）。患者与对照组在甲状腺过氧化物酶抗体和甲状腺球蛋白抗体方面无显著差异（患者的平均血清甲状腺过氧化物酶抗体为22.45±4.32，对照组为22.45±3.21，p值为0.98；患者的平均血清甲状腺球蛋白抗体为12.32±2.65，对照组为12.99±2.34，p值为0.76）。
结论
部分SCD患者可能出现甲状腺激素缺乏。
建议
鉴于SCD患儿更容易发生甲状腺功能减退且可能需要替代治疗，建议定期对其甲状腺功能进行评估。 164 ng/dL，p值为0.047；游离T4与血清铁蛋白的相关性r = -0.008，p值为0.973；TSH与血清铁蛋白的相关性r = -0.028，p值为0.9；T3与血清铁蛋白的相关性r = -0.259，p值为0.27）。患者与对照组在甲状腺过氧化物酶抗体和甲状腺球蛋白抗体方面无显著差异（患者的平均血清甲状腺过氧化物酶抗体为22.45±4.32，对照组为22.45±3.21，p值为0.98；患者的平均血清甲状腺球蛋白抗体为12.32±2.65，对照组为12.99±2.34，p值为0.76）。
结论
部分SCD患者可能出现甲状腺激素缺乏。
建议
鉴于SCD患儿更容易发生甲状腺功能减退且可能需要替代治疗，建议定期对其甲状腺功能进行评估。
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